Anemia It is a disease due to loss of ozygen carrying capacity
Polycythemia vera It is a xondition where there is increased rbc count
reflecting increased circulating rbc mass and a condton that leads to hyperviscocity Coulter counters It is the first automated cell counter Hematocrit It is the ratio of the volume of packed rbcs to the volume of whole blood WBCS, PLATELETS Buffy coat contains what 2? Making it excluded from hct examination Mcv Rbc indices that reflects rbc diameter on a wright staained blood film Mchc Rbc indiices tjat reflects the rbc staining intensity and amount of central pallor MCH Rbc indices that expresses the mass of hemoglobin and parallels the MCHCH Picrograms MCH is reported in ? RDW A 4th rbc index that expresses the degree of variation in rbc volume Unequal size Anisocytosis is whhat Different shapes Poikilocytosis is what? Polychromatophilic erythrocytes/diffusely basophilic It is a cell that indiicates the ability of the bone marrow eeythrocyte/reticulocyte to increase rbc production in aneemia due to blood loss or excessive rbc destruction True blood cells Platelers are what? Plt ct, MPV, PT, PTT, TT, Fibrinogen assay, S dimer assay 7 high volume coag tests Specimen accession It is the term for registering the specimen in the work list 62% Minimum % alcohol of alcohol gels 70% isopeopyl alcohol Most. Common skin antiseptic for venipucnture True The veins in the inner wrist should never be used due to the high risk of injury to the tendons and nerves in the area (true or false) Below Blood should be drawn below the IV with thhe torniquet also ___ the IV site Bilirubin, beta carotene, vitamin A, porphyrins Blood sensitive subtances thhat false decrease when exposed to light Ammonia, lactic acid, PTH, Gastrin 4 substances that shoild be chilled and not frozen Glucose (dec), potassoum (inc), LD (inc) 3 analaytes thar affected whene cell is not seperated from the serum within 2 hrs Parcentric It is the ability to center a cell in question in the microscopic field and rotate from one magnification poeer to another while retaining the cell cloee to the center of the viewing field Heterochromatin Two types of chromatin Euchromatin Heterochrommatin Type of chromatin that is more darkly stained, condensed clumping patternm and transcriptionally active area of the nucleus Euchromatin Type cjromatin that is pale blue, diffuse, uncondensed, open chromatin and TRANSCRIPTIONALLY ACTIVE Stromql cells Regulation of the hematopoietic microenviroment is Growth factors provided by what? Endothelial cells, adipocytes, macrophages, 7 types of stromal cells lymphocytes? Osteoblasts, osteoclasts, fibroblasts/reticular adventitial cell G1 Period of cell growth and synthesis of compoentns necessary for replication S stage Stage where DNA replication takes place G2 Stage where the tetraploid DNA is checked for proper replication and damage Mitosis / M stage Stage invovvles the division of chromosomes and cytoplasm into two daughter cells Interphase Term used for thr non mitosis stages or thr cell cycle G1, S, G2 3 stages thst belong to itnerphase Necroosis It is a pathologic process caused by direct external injury to cells Swellin of the cell First morphologic mabfiestation of necrosis Apoptosis It is a self inflcted cell death originating from the activation signals eithin the cell itslef Shrinkage of the cell Morphologic manifestation of apoptosis Extrinsic Activation of apoptosis occurs through whhat 2 Intrinsic pathways Caspase Both pathways of apotosis involved the activation of proteins called what Extrinsic pathway/death receptor pathway Pathway of apoptosis that initiates with the binding of ligand to a death receptor on the cell membrane Intrinsic pathway Pathway of apoptosis that involves release of cytochrome C from the mitochondria Mesoblastic phase Phase of hematopoieissi where development is intravascular Hepatic phase Phase of hematopoisesis that begins at 5th to 7th week and clusters of erythoblasts, granulocytes, and monocytes cam be seen in the fetal liver, thumus, spleen and placenta Hepatic phase Phase of hematopoiesis where development occurs extravascularly Hgb f Predominant hgb at birth 6 months Hb A1 starts to be predominant at what month after birth? Red marrow Component of marrow that consist of the developing blood cells and progenitors Yellow marrow Component of marrow composed primarily of adipocytes Erythroid islands Erythropoiesis typically occurs in what part of thr marrow? Erythroid islands These are macrophages surrounded by erythoid prefursors in various stages of development Megakaryocytes It is a cell that adjacent to vascular sinus Mature myeloid cells It is a cell that located deep within the cords and more mature ones are close tonsinus Kuppee cells 2 cells in yhe liver that remove sensecent cells and Macrophages foreign debris from the blood that circulates through the liver Spleen Largest lymphoid organ in the body that funx as an indoscriminate filter of the circulating blood 350ml How many ml of blood per minute passes through the spleen Hematopoietic stem cells It is a pluropotent structure that gives rise to differentiated progeny and capable of self renewal CMP, CLP 2 kinds of committed progenitor cells CMP Kind of progenitor cell that differentiates into insivodual granulocytic, erythrocytic, monocytic and mmegakaryocytic lineages CLP Kind of of progenitor cells that proliferates and differentiates into T, B and natural killer lymphocyte and dendritic lineages 1:1000 Ratio of HSC to nucleated blood cells Kidneys, liver (minor) EPO is produced by what 2 organs? BFU E Progenitor cell that has few EPO receptors CFU- E Progenitor cell that has many EPO receptors Liver TPO is principally peoduced by what organ? RBC It is the cell responsible for trasnporting nitric oxide and buffering ph 1 week It takes how long for BFU-E to mature to CFU-E 1 week It takes how long for CFU-E to mature to precursor cell 6-7 days It takes how many days for the precursors to bevome mature rbc Pronormoblast Earliest morphologically recgonizable erythrocyte precursor Decreases As the RBC mature NC ratio ______ Decreases As the rbc matures. Its overall diamter ____ Condensed As the rbc mature. Nculear chromatin pattern becomes ____ Disappear As the rbc mature the nucleoi ____ Basophilia As the rbc mature the cytoplasm loses its ___ 1-2 Nucleoli of pronormoblast Pronormoblasys Stage of erythropoiesis where the glovin productuon begins Basophilic normoblast Stage of erythropoieissj where the nuceloli present early and disappear later Polychromatic normoblast First stage in which pink color associated with hgb can be seen Polychromatic normoblast Last stage of rbc that capable of mitosis Ortichromic normoblast Stage of rbc wjere the nucleusbis extruded Pyrenocytes It is an enveloped extruded nucleus cell in the BM that is englufed by BM maxrophages 1 day Reticulocytes resides in the BM for how longg? And 1day thhe moves into the peripheral bloo for about how long? Before reaching maturity Reticulocyte Last stage of hgb production 6-8um Size of rbc Rythron It is the name given to thebcollection og all erythrocytes throughout yhe body Rbc mass Refers only to theecells in circulation 1% Approx hownmany % of rbcs normally diea each day Erythropoietin It is the prrimary oxygen sensing system of the body thatt is located in periturbular fibroblasts of the kidney Hypoxia It iis the condition that increases EPO production in peritubular cells mainly by TRANSCRIPTIONAL REGULATION True hormones Refers to the hormones that produced at one location and acting at a distant location and it is a growth factor as well Apoptosis Major way in which EPO increases rbc mass Chemiluminiscencr EPO canbe measured by what? Anemia Increased amounts of EPO in the urine are expected in most patients with ___ Directly Testosterone ____ stimulates erythropoiesis Indirectly Thuroid and pituitary jromones ____ stimulates erythropoieisis Macrophage Major cellular anchor for the rbc Fibronectin Rbc are anchored to the extracelullar mateix of the bone marrow chiefly by what? Macrophages / extravascular hemolysis It isbthe major way in which rbcs dise normally? Intravascular hemolysis Type of henolysis that due to vascular sustem that is traumatic for rbcs resulting to fragmentation Haptoglobin, hemopexxin 2 proteins used to salvage the released hemoglobin so that its iron is not lost in the urine EMP, HMP, MRP, RLP 4 major pathways of erythrocytes EMP Pathway of rbc that use glucose to generate ATPB EMP It is an anerobic glycolysis pathway of rbc where the energy is used to maintain rbc structure 2 How many net gain of ATP molecules produced by Emp HMP It is an aerobic or oxidative glycolysis pathway that detoxifies peroxide and other oxygen byproducts to protect the cell HMP Pathway of rbc that diverts glucose 6 phosphate to ribulose 5 phosphate through G6PD NADPH It is the one produced in HMP to reduce gluthatione 5-10% How many % of gluvose is diverted to the HMP Cytochrome B5 reductase HMP is aaka what MRP It is the pathway where Mechanism is to reduce ferric iron back to ferrous iron Methemoglobin Eebofbhemoglobin that is a conbiantion of hgb with. Ferric ion Rapoport leubering pathway Pathway of rbc that produces 2-3 DPG 2,3 DPG Is the one that regulates oxyfen delivery to tissues by competing with O2 fornthe oxygen bindign site of hgb True True oor false. Rbc has the property of deformability True Rbc deformability depends not only on rbc geometry but also on relatige cytopasmic viscocity. True or false Internal viscocity As MCHC rises this is also rises? Constant The ratio of cholesterol to phospholipids should be _____ Phosphatidylcholine and spingomyelin Oute layer of rbc (2 composition) Phosphatidylserine and phosphatidylethanolamine Inner layer of rbc (2 composition) Glycocalyx It is the layer of carbohydrates whose net negativ echarge prevents microbial attack and protects the rbc from emchanical damage caused by adhesion to neighboring rbcs or to the endothelium Transmebrane (integral), cytoskeletal (peripheral) 2 types of rbc proteins Blood geoup antigens, ankyrin or protein 4.1, aquaporin 3 examples of transmembrane proteins 1 Spectrin Most abundant cytoskeltal or peripheral protein Na , K, Ca Rbc membbrane is impermeable to what 3 cations? Water, HCO3, CL Rbc membrane is permeable to what 3? Which freely exchange between plasma and rbc cytoplasm Hemoglobin It is the susbtance that compromise approx 95% of the cytoplasmic cotnent of rbc 2 polypeptides Hgb contain how many pairs of two different 4 heme groups polypeptides? And how many hem groups? 4 Hgn carry up to how many molecules of oxygen? Glycated Small percentage of hgb A is what Hgb A1c 4-6% of hbA circulates in the form of what Cytoplasm and mitochondria Heme biosynthesis occurs in the what 2? Tranaferrin It is a plasma proteij that carries iron in the ferric form to developing erythroid cells True True or false. A and b chains are produced in equal amounts A chain It is the chain of hgb that has the highest affinitnfor b chain due to its negative charge Hgb F It is the hgb that compromise 1-2% of the total body hgb but only in some cells Hgb A It is the predominant hgb at 6 months of age Hgb A2 and HgbF Small amount so what 2 hgb present at 6 motnhs of age A2b2 Hgb A1 chain compoent A2D2 Hgb A2 chain compoent Glycine He key rate limiting step in heme syntehsis is the initial Succinyl coa reaction. Of ___ and ___ to form ___ catalyzed by ALA ALA synthase P50 Amoutn of oxygen needed tonsaturate 50% of hgb is called what True Theree is low hgb affinity for o2 at low o2 tension and high affinity for o2 at high o2 tension(true or false) Bohr effect A shify in the cirve due to a chabge in ph (or hydrogen ion concentration) is termed as what Myoglobin It is the one that binds oxygen with greater affinity than hgb Hgb F It is the hgb that causes a left shift due to increased oxygen affinity Methemoglobin, carboxy hgb and sulfhemoglobin 3 types of dyshemoglobins Methemoglobin It is a an hemoglobin with ferric ion and cannot bind O2 <25% Methemoglovin level that is asymptomatic 30% Methemoglovin level that causes cyanossis and hypoxia >50% Methemogobin value that causes coma and death Intravenous methylyne blue It is the one administered for methemoglobin 630nm Absorption peak of methemoglobin Carboxyhgb It is the dyshgb yhat has combinatikn of carbon monoxide with heme iron Carboxyhgb It is an hgb that has 240x greayer affinity than oxygen Carboxyhgb It is a dyshgb that is termed as silent killer becauee it is odorless and cllorless and victims quickly befom3 hypoxic 540nm Peak of carboxyhgb 20-30% Toxic effects of carboxy jgb appear at what cocentration >40% Value of carboxyhgb yhhat causes coma >3% NON SOMOKERS Carbon monoxide is diagnosed if non smokers has what >10% smokers value? And smores has what value? Sulfhgb It is a dyshgb that is irreversible and formed by addition of sulfur atom 630nm Peak of sulfhgb Cyanmethemoglobin Reference method for hgb assay 540mm Absorbtion peak of cyanmethemoglobin Sodium laryl sulfate Many isntruments now used this to convert hemoglobin to SLS methemoglpbin 65% How many % of iron found in the hemoglobin 25% How many iron is in storage within the macrophages and hepatocytes 10% How many % of iron found in the muscles, plasma and enzymes Functional conpartment 3 typed of compartments of the iron Storage comtment Transport compartment Funcyional compartment Type of compartment of iron where all iron functioning within cells Hemoglobin, myoglobin, cytochromes 3 examples of functional compartment Storage compartments Type of comparmtent where iron is nnot currently functioning but available when needed Macrophages, hepatocytes 2 cells that can functio. As storage compartment of iron Transport compartment Type of compartment that transit in the plasma Absorption The tootal amoitn of iron availavle to all body cells, systemic body iron, is regulated by what? Ferroportin It is the only known protein that exports iron across cell membranes Hepcidin It is the oneused to regulate absorption by ianctivating ferroportin Ferrous form Iron must be in what form to be absorb by enterocytes Apotransferrin/2 iron It is the one that carries iron to the plasma and can binds how many iron? Apoferritib Ferric iron is stored in a cage lime protein called what 4000 irron molecules Apoferritin can bind how many iron molecules? 1-2 mg Though 10-20 mg/day of iron is needed by thenbody. Obly how manu amount of iron is absorbed? Serum iron , TIBC, % Transdeeein saturion 4 routine tests for iron Prussian blue staining Colorimericallly Serum iron is measured how ? Serum iron Test fooriron where iron is rrleased from transferrin using an acid thrn allowed to react with the reagent Fssting Patient shoulf br what for serum iron and is collected early morning TIBC It is an indirect measure of transferrin True True orr false. A convinient rule of thumb evifent from the table is that about one third of transferrin is typically daturated with iron Prussian blue staining It is the gold standard for asessment of iron Potassium ferricyanide Prussian blue staining uses what? As ghe reagent^stain Prusian blue Is a staain that produces dsrk blue dots in tisue Ferritin It is the one that not detected by prussian blue stain Hemosdierin Prusssian blue is a stain for what Macrophages, enterocyte Ferritin is secreted by what 2 ? Ferritin levels It is the good measure of stored iron and an acute phase reactant Immunoassay Soluble transferrin receptor (sTfR) is detect with whar? Iron deficiency Increased TfR is caused by what? Thoma plot Hemoglobin conc of reticd and dtfr/log ferritin levels are plotted where? Zinc protoporphyrin It id the one that accumulates in red blood vellls when iron is not incorporated into jeme and zinc instead to protoporphyrin IX Anemia It is a condition due to decreased in the oxygen carrying capacity of the blood Anemia It is a condition that can arsie if there isninsufficient hemoglobin or the hgb has impaird function Bloood, muscle, heart In severe anemia, blood is preferentially shunted to organs that are key to survival, including what 3 ? Sudden loss of Blood volume Anemia that causes increase heart rate, respiratory rate and csrdiac output Sudden losss of blood volime Anemia that causes redistribution of blood flow from skin and viscera toheart, brain and muscle Onic bloddd loss Anemia that causes decrease in hgh oxygennaffinity by increasing the puction of 2-3 Bisphosphoglycerate (BPG) Chronic blood bloss Anemia that causes increase EPO production by the kidneys Ineffective erythropoiesis 3 mechanisms of amemia Insuufficient erythropoiesis Blodd loss and hemolysis Ineffective erythropoieisis Mechanism of anemia that due to production of erythroid precursor cells that are defective Insufficient erythropoieiss Mechanism of anemia due to decrease in the number of erythroid precursors in the BM, resulting in decreased rbc production and anmeia Cbc It is the routine test to diagnose anemia Mcv It is the most impoetant indices for anemia Reticulocyte count It is the test that serves as an improtant tool to assess the bone marrows abikity to increase rbc production in response to anemia Rpi Itt is abbetter indication of the rate of rbc production than the corrected reticulocyte count Increase Retic resutlf or avute blood loss Decrease Retic result of chronic blood loss IRF It iisa test helpful in assessing early bone marrow response after treatment for anemia Peripheral blood gilm It is a manual emthod that serves as a qc to verify the results produced by automated analyxers Oval macrocyte It is a large oval rbc that associated with megaloblastic anemia Sickle cell/depranocyte It is a thin dnese, elongated rbc pointed at each end Hgb C crystal It is a hgb with a hexagonal crustal of eense hgb Hgb SC crystal It is a finngerlinek or quarts like ctystal hgb Target cells/codoycytes/leptocyte It is an rbc with hgb concentrated in crnter amd periphery IDA/Thalassemia Target cells cam be seen in what 2? Schistocytes Helmet cell is looks like what Keratocytes Helmet cell is aka what Folded cell It is a cell where its membrane is folded over Hgb C/SC Folded cell can be seen in what 2 hgb? Acanthicyte It iis an rbc with irregularly spaced projectione Acanthocyte It is a poikilocyte seen in severe liver disease Acanthocyte It is a poikilocyte yhat seen in abetaliporpoteinemia and mcleod syndrome Spur cell Acanthocyte is aka what Burr cell It is an rbc with blunred short projections that are evnly spaced Burr cell It is a crenated rbc yhat csn be an artifactual Echinicyted Burr cell is aka what Myelofebrosis Teardrop cell/dacrocytes can be seen in what 3? Myelopthisic anemia Pernicious anemia True Diffuse basophilia is rna and found in retivulocytes. (True or false) Basophilic stipplings It is an precipated rna that can be seen in hgbpathies DNA Howel jolly bodies structure Heinx bodies It is an ebc inclusion that due to denatured hgb Pappenheimer bodies It is an rbc inclusion composed of iron that can be seen in sideroblastic anemia Mitotic spindle Structure of cabot rings B globin chains Composion of hgb H BM examination It is the one thst idnciated for a patient with an unexplained anemia associated with or without other cytopenias, fever or unknown origin or suspectrf hematologic malignancy Mcv It is an extremenly important tool and id keu in the morphologic clasification of anemia Micro hypo Hgb H dsee is what type of anemia Megaloblastic anemia Examplles of macorcytic anemia Alcoholism Liver diseae Rdw It is a lab test thatt can help determine the cause of an anemia when used in conjiction with the mcv Cbc, retic count, pbs 3 intial lab test for anemia Stage 1 Staage of IDA that is latent or sublinciaal thst idnciates gerritin is low and asymptomatic Stage 2 Stage of IDA where storage is exhausted and hgb of retics begin to decrease Stage 3 Stage of IDA classified as frank anemia and characteristed by gloossitis, koilonychia and pica Trichuris tirchuria, schistosoma mansoni, schistosoma Aside from hookworm. Thiis 3 parasites also causes iron haematobium deficiency >15% An RDW greater than this value is expected and may precede the decrease in hgb Iron studies It is the backbone for diagnsois of iron defincirncy Serum iron, TIBC, ttansferrin saturation, serum ferritin 4 iron studies that used for IDA dx 2 days During txt. Reticulocyte hgb content will correct within how many. Days? 5-10 days During txt. Reticulocyte beings to increase within ? 2- 3 weeks During txt. The anticipated rise of hgb appears begins how many weeks? Transferrin Iron is indirectlly proportional to what? Decreases Transferrin resultnif there isbinfection Anemia of chronic inflammation In this type of anmeia. There is impaired mobilizarion Sideropenia Central feature of anemia of chronic inflammation Hepcidin During inflammation. The liver increases the syntheeis of what? Hepcidin It is an nom specific defense against invading bacteria during inflammation Lactoferrin It is an iron binding protein in the granules ofnenutrophilis Transferrin Lactoferrin avidiy for iron isngreater than what? Sideroblastic anemia Itbiss an anemia that prevents production of protoporphyrin or incorporation of iron into it Prussian bluenstain Stain can be diagnosed sideroblastic ameia Ring sideroblaats Hallmark of sideroblaatic anemias Lead poisoning In thisncondition peripheral neuropathy cam be seen Lead It is a susbtance that interfers porphyrin synthesis via conversion of ALA to PBG by ALA dehydratase leading to accumulation of ALA Heme synthase Ferrochelatase is aka what Prophyria It is an hereditary condition that impair production of protoporphyrin and photsensitivty is the greatedt problem Ferritin Bodys first reaction is tonstore excess iron in the form of what? Hemosiderin It is an abnromal iron storage form that can lead to hemochromatosis Bronze diabetes Iron overload is aka what ? Megaloblastic anmeia It is an anemia affecting the dna metabolism 5- methyl THF Folate circulates in the blood predominantly as what Folate deficiency, vit b12 def 2 typess of megaloblastic anemiias Folate def Type of megaloblastic anemiia that has more direct effect Vit n12 def Type of megaloblastic anemia that has more indirect effect that prevents production of THF from 5-methyl THF Folate When vit b122 is deficient. It is the one that more anore produced and befime trapped as 5-methyl THF Nuclear-cytoplasmic asynchrony The slower maturation rate of the nucleus compared with the cytoplasm is calle dwhat Dysplastic erythroid cells, CDA type 1 and 3, FBA M6 3 other causes of emgaloblastic anemia Type 1 Type of CDA that has internuclear chromatn bridging of erythroid cells or binucleate dforms are observed Type 3 Type of CDA that has presence of giant multinucleated erythroblasts Imapried use of drugs (chemo) 2 other causes of folate and vit b12 def Excessive loss (dialysis) Pernicious anemia, H. Pylori infection, lack of intrinsic 6 causes of vit b12 def factors, gastrectomy, d latum, achlorhydria CBC, retic ct, WBC manual diff ct, serum bolorubin, LD 5 tests uused to screen megaloblastic aneemias Oval macrocytes In vit b12 ded. There is presence of what ? Decreased Retic ct result of vit b12 def. There is no polychromasia and has pancytopenia Hypersegmented neutrophils Present in wbc differential of megaloblastic anemia Increaaed Bilirubin and LD result of megaloblastic anemia Deceeased Vitamin Levels, hypersegmentation, oval 6 sequence of development of megaloblastic ameia macrocytes, megaloblasrosis in BM, anemia Immunoassays Folate and b12 levels can be measured using what? Chemiluminscence B12 can also measure via what Deoxyuridine suppresion test, stool analysis of 2 other tests for meglaoblastic anemia paraaites 1 week After txt. During thiis week reticulocyte response is substantial 2 weeks After txt. Dueing this week hypersegmentation disappears 3 weeke After txt. During this week. Hgb increaaes towards normla Non megaloblastic It isna type of amcrocytin anemia where the dna synthesis is not impaired Non megaloblasric anemias Type of macrocytic anemia that has no hypersegmentation of enutrophils, no oval macrocytes and no megaloblasts in the BM Newborns, retiiculocytopenia, liver dse, Chronic 5 causes of non megaloblastic anemia alcoholism, BM failure BM failure amemias It is a condition where there is reduction or cessation of bloood cells prpduction affecting one or more cell lines Acquired PNH, autoimmune dsieases and pregnancynis what type of aplastic anemia Fanconi anemia 3 types of inherited aplastic anemia Dyskeratosiis congenita Swachman bodian diamond syndrome Erythropoietin In apllastic anemia. There is elevated elvels of wjat 3 ? Thrombopoeitin CSF Normo normo Type of anemai present in aplastic amemia Aplastic anemia Type of anemia characterized by hypocellula rbone marrow and can undergo biopsy Inc Ironr esult of aplastic anemia Monosomy 7 2 most common cytogenetic abnormalities of aplastic Trisomy 8 anemia Fanconi anemia It is a chromsomal instability disorder cahracterized by cancer susceptibikity Chromosme breakage analysis Diagnostic test for fanconi anemia Dyskeratosis congenita Type of aplastic anemia charwcterized by mucocutaneous abnormalitied Dyskeratosis cingenita Type of aplastic anemia characterized hy abnormal skin penetration, dystrophic nails and oral leukoplakia Shwachman bodian diamond sydnrome Type of aplastic anemia characterized by pancreatic insuffciebcy and predisposition for hematologic malignancies Pure red cell aplasia Type of aplastic anemia characterized by selective and severe decrease in erythrocyte precursors Pure red cell aplasia Tyoe of aplastic anemia charwcterized by severe anemia and reticulocytopenia wirh normal wbcs and platelets Transient erythroblastopenia of childhood (TEC) 1 ex or acquired pure red cell aplasia Diamond blackfan anemia 1 ex of congenital pure red cell aplasia True True or false. CDA has hypercellular BM but ineffective erythropoeisis 3 How many types of CDA we have CDA 1 Type or CDA that hae spongy heterochromatin wirh swiss cheese app. CDA 2 Most common subtype of CDA HEMPAS 1 ex of CDA 2 Sucrose hemolysis test HEMPAS/CDA 2 is negative to what test Cda 3 Least common CDA wheree BM has megaloblasric changee. And has characterisric feature of Guant erythroblaats with up to 12 nuclei Myelophtisic anemia Type of aplastic anemia that has infiltration of abnormal cells into bone marrow and subsequent destruction and replacement of normal hematopoetic cells Tear drop eeythrocytes 2 charwctersircs of pxts with myelopthisic anemia Hypercellular BM Anemia of chronic kidney disease It is condition thwt has complication of renal dse and inadequate renal erythropoetin Uremia It is the one thqt inhibits erythropoieiss and increases rbc fragiliry Burr cells Anemai of chronic kidney disease poikilocyte Thalassemia It is a conndition caused by genetic mutations that reduce or prevent the synthesis of one or more of the globin chains of the hgb tetramer Anemia, hepatosplenomegaly, mongolid faces 3 features present in thalassemia Thalassemias It is a condition thwt has high incidence in mediterranean descent True True oe false. In thalassemia. There is imbalance of a/b chain ratios Thalassemia minor (heterozygous thalassemia) Type of thalassenia that imparts resistanfe to malaria 16 Alpha and zetaglobin chain is found on what chromosome Beta, gamma, epsilon , delta 4 globin chains found in the chromosme 11 Hb A1 Hgn that found 95-100% in adults Hb A2 Hgb found 0-3.5% in adult Hgb F Hgb found 0-2% in adults and common in fetus B/B Normal B chain synthesis genotype Aa/aa Normal A chain synthesis grnotpye 11 B thalassemias include l disoders ariising from the B glovin gene cluster whar chromosome? 16 Thalassemias include ALL disorders from what chromosome Micro/hypo Thalassemia stems can result tonwhat typenof anemia Yhaalsdemia It is a conditikn due to unequal production of globin chains eesulting in accumulation of one type which decreases survival of rbcs and precursors Gamma chains In fetus and newborns, lack of a chains lead to exchess what chain? 4 gamma chains B bart genotype Beta fhains Afte 6 months, there is switch of gamma chain to beta leading toe xcess what ? 4 beta chains Hgb H genotype Moderate hemolytic anemia As rbc matures? Hb H ppt. And forms inclusion bodies which is removed extravascularly by the spleen leading to what? True True or false. Hgb bart and hgb H cannot deliver oxygen to tissues due to their very high affinity for oxygen Heart failure, massive edema, hydrops fetalis Hgb bart and Hb H can reeult to what 3 diseases of fetus Hgb barts T is thee hgb that can lead to fetal death Hgb H It is an hgb thwt has moderate anemia and is transfusion independent Constant spring (a^cs) It is an hgh that result of a point mutation that changes the stop codon at 142 to a glutamine codon with an additional 31 amino acids added to the C-terminal end B thaalsdemia Type of thalassemia that has premature death of rbccs in the bine marrow B thalasdemia Typenof thalassemiabwhere skull radiopgraphs may demonstrate typical hair on end and there is prominence of forehead, cheek bones and upper jaw Gamma and delta chains What 2 chains is increase to compensate B thaladdemia B silent Type of b thalassemia that has minimal reduction in production
Leopre Tyoe of b thalasshemia that indicates fusion of the
deltabin. Genes Leopre Is the term where there is crossover between the d globin locuson one chromosome and b globin locus on the other chromosome B+/B+ , B°/B° , B+/B° , lepore/lepore Enotypesnof b thalassemia major (4) B thalassemia major Ppe of b thalassemia thatbcharacterized by severe anemia that requires regular transfusion, and leads toe xcrss iron burden Deferoxamine Remedied for excess iron in b thalassemia major Bsilent/Bsilent , B+/Bsilent, B°/Bsilent, SB°/SB° , B°/SB° 5 GENOTYPESNOF b yhalassemia intermedia B thalassemia intermedia Typenof b ththhat is more severe yhan minor but does not require regular transfusions B/Any Genotype of B thalassemia minor B thalassemia minor B thalassemia minornis aka what? Which is asymptomatic anemia Silent carrier state Type of b thalassemia that is Bsilent/B and small decrease in production of beta chains Cbc, bloodd smear, iron studies 3 screening test for thalassemia tonrulenout IDA Target cells Poikilocyte common in thalassemia Suoravutal stain, hgb fraction quantification by 2 presumptive test for thalassemia electrophoresis Hgb H It is an jgn yjat produce a pitted pattern on the rbcs similar to the patternbof a gold ball or raspberry Molecular genetic test Definitive test for thalassemia Hgb electrophoresjs at alk. Ph Traditional tool for hgbnipathy and thalassemias Agartrophoresis acid ph, HPLC, CZE 3 methods used for confirmation of thalassemia Alkali denaturation test It iis an accurate and precise test tonquantify hgb F in the 0.2% to 50% range. Hgb F Itnis an hgb that is not denatured by alkali denaturatikn test Ethanol Fixative in kleihauer betke acid elution test 3.5 Buffer ph used in kleihauer betke Hgn F It id the hgb that resist elution of kleohauer betke Pink Hgb color in kleihauer betke Ghist cells Hgb appearance in kleihauer betke Decreased Osmotic fragilittest result of pxt with thalassemia Iron studies It is abtest that best differentiated iron deficiency and b Hb A2 level , Cbv and blood smeae thalassemia minor (4) Hemoglovinopatheis It is a diseases state involving the hgb molecule Hemoglobinopathies It is the most common genetic diseases POinnt mutation It isnthe most common type of genetic mutation occuring in the jemoglobinopathies Zygosity It refers to the association between the mumber ofngene mutations and the level of severity ofnthe resultant genetic defect Hgb S , unstable hgn (HbKoln) 2 abnormal.hgb yhat can result in hemolytic amemia Hgb M It is an abnormla hgb that result in methemoglobinemia Hgb kaansas 2 hgb that has either increased or decreased oxygen Hgb chesapeake affinity Plasmodium falciparum Sickle cell confers resistance to what? Reversible sickle cells 2 forms of hemoglobin S sickle cell Irreversible sickle cells Reversible sickle cells The vasoocclusive complications of SCD are thought to be due to what? Hgb F Symptomps of sickle cell dse is free until until the second half of the first year of life because of the protective effect of what hgb? Crises Individuals with thisdisease undergo episodes ofnrecurring pain termed what ? Vasoocclusive crises Hallmark of Sickle cell dsiease Autosplenectomy Gradual loss of function is reffered as what ? Acute infections It is the common cause of hospitalozation of people with SCD and have been the most frequent cause of death espcially in thebfirst 3 years of life Normocytic normochromic The anemia of SXD is cheonic hemolytic anemia classifed morphologically as what ? Hgb S (sickle cells disease) Hgb S 80% and Hgb F 1-20% what is the hemoglovinopathy Target cells 2 hallmark of SCD in terms of poikilocyte Sickle cells Hemoglobin solunility test ( dithionite tube test) Most common screening test fornhgb S Turbidity in solution Decreases solubulity of deoxygenated hgb S caues what? Saponin, reducing agent 2 solutions used in hgb solubitlity test tonlysed rbc Dithionite 2 reducing agent used in hemoglobin solunility test Sodium hydrosulfite Reducing agent Rgnt in sodium solubility test that used to reduce iron to its ferric form converting the hgb tonits deoxygenated form Sodium metabisulfite test Old screening test for hgb S 2% sodium metabisulfite Rgnt used in sodium metabosulfite test to induce sicle cell formation Sickle cell/ holly leaf formatikm + result in sodium metabisulfite test Alkalinr hemoglobin electrophoresis It is the common first step in the confirmation of hgbnipathies including SCD True True or false. Supportive care has been the mainstay of therapy for SCD Handfoot syndrome Chilldren younger than 3 years with SCD often experience what? Sickle cell trait It is an heterozygous, benign condition of sickle cell that only in extreme low oxygen condition Sickle cell trait <40% hgn S and 60% Hb A1. What is the hemoglobinopatheis? Sickle cell trait Failure tonconcentrate urine is the only consistent abnormality found in pxts with what? Hgb C It is the most common non sickling vsriant of hemoglobin encountered in the united states Hgb C It is the 3rd most common hemoglobinopathy in the world Hgb C It is an hgb that has polymers of a short, thivk crystal within rbcs Hgn C Itnis an hgb that has presence of hexoganl crystals or can be that the hgb is concentrated within the boundary of the crystal Wet preparations These crystals of hgb C may be seen on what? Sodium vitrate Wasshing rbcs and resuspending them in a solution with wha Hgb E It is an hgbpathy that is high prevalence in south east asia Microcytic rbcs Size of rbcs in patients with hgb E hemoglobinopathy Hgb SC It is the most common heterozygous sundrome of hemoglobinopathy Hgb M It is an hemoglobinopathy that has variety of mutations that result in production of methemoglobin Unstable hgbs It is an hgb that products that precipitate in vivo, producing heinx bodies and vausing a hrmolytic anemia Isopropanol precipitation test 4 tests for unstable hgbs Heat denaturation test Heinz body staining technique Peptiide analysis – to confirm Isopropanol This rgny at 37C weakens the bonding forces of the hgb molecule Ppt at 5 mins l, flocculation at 20 mins Unstable hgb result in isoprooanol precipitation test 40 mins Normal hgb precipitates at what time? 50C within 1 hr In heat denaturation test whenincubated at thisntemp within what time?, heat sensitive unstable hgbs show a flocculent ppt Hemolytic anemia Itbis a condition that results when the rate of rbc destruction exceeds the increaaed rate of rbc production Hemysis of mature rbc It is the primary cause of anemia Acute Type ofhemolytic anemai that is rapid onset and appears and disappears between episodes PCH/PNH/HTR 3 examples of acute hemolytic anemia Chronicnhemolytic anemia Type ofnhemolytic anemai that may not be evident but can cause crisis over time G6PD def 1 example of chronic hemolytic anemai Abetalipoproteinemia, LCAT defiiency 2 examples of inherited hemolytic anemia PNH 1 example of acwuired hemolytic anemia Intrisnic It is an jemoyltic anemia due to abnormalities of the rbc membrane, enzymatic pathways, or the hemoglobbin moelcule Intrinsic Type ofnhemolytic anemia that rbcs are abnormal so it cannot be donors and only recipient and most are inherited Exteinsic It is an hemolytic anemia that arise from the ourside ofnthe rbc and substances conditions affecting the circulatory system Immunohemoyltic, non immune Extrinsic can be what 2 ? Extrinsic It is an hemolyric anemia that cannot be recipients and only donors. Mosy arr acquried Macrophages Extravascular is fue to what Intravascualr Fragmentation fornwhat type ofnhemolysis Extravascular Macrophage mediated for what type of hemolysis Bilirubin It is an. Rbc breakdown products Recycled Hemoglobin will break down to heme and globin. What happened to globin Recycled Heme will bebdegraded into biliverdin + iron. What happen to iron Haptoglobinhemopexin-methemalbumin system It is an system that exists to salvaged hemoglobin iron Haptoglobin It is the one responsible for the salvage of free hgb ehrre both are degraded by the reticuloendothelail system Hemopexin It is the one responsible for the secondary mechanism of iron sllavage and oxidation prevention that is bound by metheme Metheme-albumin system It is the third mechanism of iron salvage True or false. If the previous systems are overloaded, the excess hemoglobin and metheme will be filtired into the urine causing hemosiderosis and hemoglobonuria Extravascular hemolysis Poikilocytosis and inclusion bodies can cause what? Haptoglobin Both intravascular and extravascular have both Hemopexin decreased in what 2 ? Erum free hgb Both extra and intravascular have characterised by the increased of what InTravascular Typenofnhemoylsis characterized by presence of urine free hgb and urine methemoglobin Intravascular Type ofnhemolysis that has presnet prussian vluenstaining ornurine sediment Schsitocytes Poikilocyte present in intravascular hemolysis Spherocytes Poikilocyte present in extravascular hemolysis Kernicterus Hemolysis effectnin newborns>20mg/○♧ <1mg dl Normal physiologic fragmentation hemollysisbproduces a plasmahgb level of what? 50 mg/dl Hemoglobinlevel that can cause visibly red/brown plasma Carbon monoxide It is the one produced in the first step of heme breakdown by heme oxygenase Hba1c 2 chemiistry test that can also be used to detect LD hemolysis. Reticulocytosis is also present Vertical structure integrity 2 types of membrane abnormalities Horizontal structural integrity Vertical strutcural integrirty Type of membrane abnormality that provided by ankyrin complex and protein 4.1 complex that acnhors the lipid bilayer to the underlying cytoskeleton Horixontal structural integity Type of membrane abnormaloty that provided by a and b spectirn that interacts laterally to form tetramers and prevent membrame from fragmentinf during stress Hereditary spherocytosis It is a group of anemias thatbhave defects in proteins that disrupts the vertical interactions Hereditary spherocytosis Type of intrinisc anemia that has decreased surface area to volume ratio, and the cells become spherical Anemia, jaundice and splenomegaly 3 clinical manifesations of hereditary spherocytosis Splenectomy It is thhe treatment that is reserved for moderate tonsevere cases of HS Pparasocopy Recommended method for Detemination of HS 35-38% MCHC and RDW redult of HS >14% Increased Osmotic fragility test result of HS because it jas decreased sueface area to volime ratios True True ornfalse. In OFT. Blood is added to a seried of tubes with increasingly hypotonic sodium chloride solutions 0.85% At thid NaCl concentration the amount of water entering the cell is equivalent to the water leaving the cell because the intracellular and extracellular osmolarity is the same 540nm In OFT. The absorbance ofnsupernatant is measured at what nm? To check for hemoylsis 0.45% Normal rbcs initial jemolysis starts at what? And ends at 0.35% and 0.30% what? 37C for 24 hrs Prior incubation at this temp for how long? Can be used to detect mild cases and also known as incubated OFT EMA binding tedt (Eosin-59-maleimide) It si a test for transmembrane proteins where HS patients show a lower mean fluorescent intensity (MFI) Ektacytometer Test for HS for variation in membrane surface area and cell water content can be determined Acid glyxerol lysis test Test for HS whee the amount of hemolysis after a patient rbc are incubated with a buffered glycerol solution at an acid ph SDS-PAGE 2 other test for HS radioimmumoassay 48 hre In autohemolysis test. Pxts rbcs and serum are incubated for how long? 10-50% In autohemolysis test. HS causes hemolydis at what Glucose or ATP percent ? Which can be corrected either by what 2? True Cells from HS patients are particularly sensitive to cooling at 0C in hypertonicbsolutions HS Ppeetoinic ceyohemolysis test is test for what? DAT It is a test to differentiiate HS from spherocytes due to immune related hemolytic anemia Negatiive HS result in DAT Hereditary elliptocytosis It is an group of anemia from gene mutations in which the defective proteins disrupt the horizontal linkages Hereditary elliptocytosis True or false. Hereditary elliptocytosis causes extravascular hemolysis and anemia but usually asymptomatic Leach phenotype Herediatry elliptocytosis is associated with what phenotype Gerbich antigens Leach phenotype lacks what 2? Glucoprotein C (GPC) Editary pyropoikilocytosis It is a severe form of He That results in marrked thermal sensitivitt 41-45C In hereditary pyropoikilocytosis, rbc fragments occurs at what temp? 49C Normal rbcs fragments at what temp? South east asian ovalocytosis Hereditary ovalocytosis is aka what? Hereditary ovalocytosis It is a condition caused by a mutation in the gene for band 3 that results in increased rigidity of the membrane (Intrinsic hemolytic anemai) Malaria Herediatry ovalocytosis confers resiistance to invasion by what? Hereditary ovalocytosis It is a type of intrinsic hemolytic anemia common in amlaria belt of southeast asia Familial pseudohyperkalemia 3 types of stomatocytes diseases Cryohydrocytosis Rh deficiency syndrome Familial pseudohyperkalemia Type of stomatocyte disease thar has excrssive potassium leaks out of the rbcs at RT in vitro but not at body temperature in vivo Cryohydrocytosis Type of stomatoocyte disease where the rbcs have marked cell swelling annd hemolysis whrn stored at 4C for 24 to 48 hrs Rh definciency sydnrome Type of stomatocyte disease where the expression of rh membrane protein is absent (rh nul) or decreased (rh mod) Neuroacanthocytosis 2 types of diseases that has presence of acanthocytes Spurr cell anemia Neuroacanthocytosis It is a group of disorders characterized by neurologic impairement and acanthocytes Abetalipoproteinemia 3 types of neuroacanthocytosis Mcleod syndrome Chorea acanthocytosis Chylo, LDL , VLDLLD 3 lipoproteins absent in abetaliporpoteinemia Abetalipoproteinemia Type of neuroacanthocytosis that had low chole and tag but sphingomyelin is increased Kx gene Mutation in mcleod syndrome Chorea acanthocutosis Type of neuroacantjocytosis chsracterized by chorea hyperkinesia, cognitive impairements, and neuropsychiatric symptoms Spurr cell anemia Type of disease of acanthocytes where pxts with sever liber disease that develops a hemolyric anemia with acanthocytosis CD55 (DAF) CD59 (MIRL) 2 lacks in PNH thst renders them susceptible to spontanrous lysos by complement Hepatic vein thrombosis Most common thrombotic manifestation of budd chiiari syndrome which obstructs venous outflow from the liver Flow cytometry Diagnostic test for PNH Sugar water test 2 old test for PNH Hams test EMP and HMP 2 most important metabolic pathways G6PD and PK deficiency 2 most commonly encountered enxymopathies G6PD It id an enxymopathies that vulnerable to oxidative damage and subsequent hrmolysis G6pd It is the most common rbc enzyme defect and confers protection against p. Facilparum and vivax in mmales Heeinz bodies 2 poikilcoyte present in G6PD and has decreased hgb Bite cells solubility and rapidly remoged by intra and extra hemolysis Type 1 Worst and severe type of G6PD Infection Most common cause of hemolyssis in G6PD Primaquine, mothballs/napthalene, favism 3 that causes G6PD Quantitative spectrophotometric asdyas Gold std tondetermine G6PD Activiy anf make a definitive diagnosis amd asess the severity of the definciency G6PD + NADP (non flouresencent) -> 6- Principle of G6PD flourescence spot test phosphogluconate + NADPH (Flouredecent) Glucose In autohemolysis test. G6PD is corrected only by what Ascorbate cyanide test It is the mst sensitive screening test for G6PD PK deficiency Type of enzymipathies that has lack of ATP formation leads to reduced deformability and maintenance of cell structure PK deficiency It is the most common form of hereditary non spherocytic hemolytic anemia and is found worldwide Qualitative tests for PK def. It isbused for screening test of Pk def. Extrinsic non immune hemolytic anemia It isa cindition ourside of the rbcs casuing premature jemolysis and is not immuen related Physical or mechanical injury of rbcs Common feafure of extrinsic non immune hemolytic anemia Microangiopathic hemolytic anemia It is a group of potentially life threatening disorders characterized by rbc fragmentation and thrombocytopenia Schistocytes Characterisitc poikilocyte seen in microangiophatic hemolytic anemia Helmet cell, microspherochtes Microangiopathic HA also produces what 2 oikilocyte TTP, HUS, HELLP and DIC 4 types of MAHA LD activity Cc parameter elevated in TTP ADAMTS-13 TTP isnusually due to deficient vwf cleaving protease known as what? Upshaw schulman syndrome Inherited TTP is called what 2? Severe ADAMTS- 13 deficiency HUS Type of MAHA characterized by microangipathic hemolytic anemia, thrombocytopenia and acute renal failure Typical HUS, Atypical HUS 2 types of HUS Typical HUS Typenof HUS that is shiga toxin associsted And more common than atypical STEC E.coli 0157:H7 and shigella Most common cause of HUS infection Atypical HUS Type of HUS caused by unregulated activation of the alternative complement pathway HELLP Type of MAHA that occurs in less than 1% of all pregnancies but develops in approx 10-20% of pregnancies with severe preeclampsia most often In the third trimester Normal PT and PTT result of HELLP DIC Type of MAHA due to widespread activation of the hemolytif sustem Thrombocytopenia Consistent findings in DIC Prolonged PT and PTT result of DIC Increased Fibrinogen and D-dimer level result of DIC Traumatic cardiac hemolyric anemia 2 types of macroangiopathicnhemolyric anemia Exercise indeccued hemoglobinuria Exercised induced hgbniuria Type of macroangiopathic hemolytic anemia where shxistocytes are notnpresent Malaria, babesia, Clostrodum, barttonella 4 organisms that can cause anemia Malaria It is annorgnamism that can cause anmeia bybdirect lysis of infected rbcs during schizogony Malaria It is an organism thst causes anemia by direct lysis of infectrd rbcs and non infected rbcs in the spleen Malaria It is an organism that causes anemia via inhibition of erythropoiesis and ineffective erythropoieisis Babbesia It is an intraerythrocytoc parasite that cauuses hemolysis Closteidium It is a bacteria that produce s alphha toxin with phospholipase C and sphingomyelinase activity that hydrolyzes rbc membrane phsopholipids Bartoenlla bacilliformis It is an small pleomorphic, intracellular coccobacillus adhheres to rbcs and cause hemolysis Dapsone and napthalene 2 drugs that causes hemolysis Rbc hemolysis Venoms causes what 49C Warming normal rbcs to this temp in vitro induces rbc fragmentation and budding Spherocytes, schistocytee, microspherocytes 3 poikilocytes can be seen in extensice burns Autoimmune, alloimmune, drug induced hemolytic 3 class of extrinsic immunne hemolytic anemia anemai PCH, CAD, WAIHA 3 typed of autoimmine extrinsic HA HTR and HDN 2 typpes of alloimmune extrinsic HA Extravascular, intravascular 2 emchanisms of immune hemolytic anemia IgG, IgM 2 abs isotype involved in immune hemolytic anemia IgM It is aan antibody that rewuires complement and can be result to both INTRA and EXTRA hemolysis IgG It is an antibody that occurs with or without compllement and predominantly by extravascular mechanisms C3b sensitized rbcs It is abcomplement that can be recognized by macropahges C3d sensirized rbcs It is a complement that cannot recognized by macrophages Spherocytes The poikilocyte seem in extrinsic jemolytic anemias DAT It is a test to determine if the hemolysis is due to an immuen mechanism of anemia IgG Ig type of WAIHA Igm Ig typenof CAD IgG Ig type of PCH Igg and IgM Ig type of mixed 37C , 4C Temp required by WAIHA and PCH 4->30C Temp required by CAD 4-37C Temp required by mixed Variable It is the only extrnisic hemolytic anemia that variable in complement activation CAD, PCH 2 type of extrinsic immune HA that occurs intravascularly hemolysis Waiha Type of extrinsic immune HA that extravascular jsis True CAD is mostly intravascular but rare extra. True or false Mixed Type of extrisnic imune HA that is both hemolysis can occur Rh complex Blood group associsted to WAIHA I (most) I some) Blodo group associated to CAD P Blood group associated to PCH Mixed Type of extrinsic immune HA that panreactive blodo group Pr (rare) Rare bloodngrouo that can cause CAD WAIHA It is the most commonly encountered autoimmune hemolyric anemia compromising 70% cases Cold agglutinins Itnis an autoantbodies of thebigm class that react optimally at 4C and more commonly found in healthy indoviduals True or false True or false. Most pathologic cold agglutinins are monoclonal? Occur at high titers and are cappable of reacting at temp greater than 30C 1:1000 Titer of cold agglutinins at 4C Mycoplasma pneuomoniae, IM, viral infections Acute CAD occurs secodnary to what 3? 37C maintainment Spx handling for CAD blood specimens 37C maintainment It is done in CAD blood specimens to prevent the biddning of the autoantibody to the patients own rbcs, which can false decrease the ab titer in the serum PCH It is an acute form of cold reactive hemolytic anemia Late stage syphilis Secondaru PCH was associsted with what ? PCH It is an disease thar now is the msot commonly seen in young childrrn after a viral respiratory infection Anti P autoantibody, donath alndsteiner ab Autoantibkdy associatedbwith PCH Anti P autoantiboodu It is a complement binding IgG hemolydin with sepcificty for the p antigen on rbcs and also a biphasic antibdoy Druf absorption 4 mechanisms of drug induced hemolytic anemia Drug rbc membrane protein immunogenic complex Rbc autoab induction Membrane modofication method IGg Ig associated to drug adsorption IgG and IgM 2. Ab produced by drug rbc memrbae protein immunogenic compelx that causes acute intravascular hemolysis Rbc autoantibody nduction It is a mechanism of drug induced hemolytic anemia where a drug induced the patient to produce IgG wsrm reactive autoantibodies against rbc self antigens causes extravadcular hemolysis True True or false. Mixed type hemoyltic anemia produces both IgG and IgM abs that can react both at 4C to 37C Accidental transfusion of ABO incompatible blood (IgM) Most common cause of AHTR Delayed HTR Type of HTR that occurs days to weeks after trnasfusion as the titer of alloantibodies increases and usually IgG Hdn Is a disease that occurs when an IgG alloantibkdy prroduced by the mother crosses the palcenta into the fetal circulation and binds tonfetal rbcs thst are positive for the corresponding antigen Macropahges N HDN IgG seensitized fetal rbcs are cleared from the Extravascular hemysis circulation by what? In the fetal spleen causes what? And an anemia gradually develops Hyydrops fetalid In HDN, if the anemia is severe in utero , it can leadd to generalized edema, ascites and a condition called what? Which is fatal if untreated O- mother In ABO incopatibility. The mother is blood type what? A or B - child And what is the blood typr of child? Rh It is a severe ttpe of HDN that has severe jaundice and anemia ABO Type of HDN that has presence of spherocytes Rh Type of HDN that is psoitive to DAT ABO Type of Hdn thar negative to DAT or weakly positive PHA It is a qualitative disorder that has decreased nuclear segmentation All leukocytes Affected in PHA PHA It is a qualitative disorder that has spectacle like (“pince-nez) morphology but the function is normal True True ornfalsem in true PHA thennumber of affected cells is much higher than in pseudo PHA Neutrophil hypersegmentatikn It is a qualitative disorder wwhere neutrophils have more than five lobes and are most often asdocisted with megaloblastic anemia Myelodysplastic syndrome, myeloid dysplasia, 4 conditions that can cause neutrophil myelokathexis, WHIM hypersegmentation Warts neutriphenia, hypogammaglobulinemia, Transcribe WHIM infrction, myelokathexis Alder reilly anomaly Type of qualitative disorder that has granulocytes with large, darkly staining metachromatic cytoplasmic granules composed primarily of partially digested mucopolysaccharides Hunter syndrome, hurler syndrome, maroteux lamy Reilly bodies are most commonly associated with 3 polydystrophic dwarfism diseases? Chediwk higashi Type of qualitative disorders that has fused lysosomal grnaules which is larfe and non functional Leukocyte dysfunction, recurrent pyogenic infections Chediak higashi may result in what 2? Chediak higashi Type of qualitatice disorder that kften have bleeding issues due to abnormal dense granules in plts May hegglin anomaly Type of qualitatice disorde that characterized by thrombocytopenia, giant platelets, and dohle like inclusions Monocytes, eosinophils, neutrophils, basophils May hegglin anomaly has dohle body like inclusion in what 4 cells? MHA Typenof qualitatice disorder where inclusions are compsoed of precipitated myosin heavy chains CGD It is a disorder that has decreased ability of phagocyted to produce superoxide and reactive oxygen species due to mutations with NADPH oxidase Nitrroblue tetrazolium reduction test Test for CGD Yellow Nornal color of the NBT reafent Blue If the Neutrophil is normal. What is the color of NBT Yellow If the person has CGD l. What is the NBT result Foormazan Gives dark blue color In NBT test Flow cytometry New test for CGD Dihydrorhodamine - 123 Flourescent prove used in CGD LAD Type of wualitative disorder that result in the inabilitt of neutrophils and monocytes to adhere to endothelial cells and to transmigrate from the blood to the tissues MPO def Type of Qualitative disorder that due to a deficiency in myeloperoxidase in the 1° granules of neutrophils and lysosomes of monocytes Dermatan sulfate, heparan sulfste, keratan sulfate, Mucopolusaccharidoses has a deficirnt aactivitt of an chondroitin sulfate enxyme necessary for the degradation of what 4 substance? Mucopolysaccharidoses It is a quualitative disorder thst results in phsysical abnormality and sometimee membrane retsrdatiom Gaucher, niemann pick, fabrys, tay sachs and sandhoffs 5 types of lipid storage disease Gaucher It is the most common of the lysosomal lipid storage diseases Gauchers disease Typenofnlipid storage disease that has defect in b glucocerebrosidase Ashkenazi jews Gauchers is common in what people Stristed or wrinkled (onion skin) Appearance of cells in gauchers PAS stains It is atest for mucopolysaccharides in gaucher cells Niemann pick Type of lipid sotrage disease thst has defiicient in sphingomyelinase and subsewuent build up of the substrste sphingomyelin Niemann pick Type of lipid storage disease that has macrophages with a foamy cytoplasm pack with lipid filled lysossomed that appear as vacuoled after staining Fabrys Type of lipidsotrage disease that has a galactosidase deficiency Hexosaminidase A Defect in taysachs Hexosaminidase A and B Defect in sandhoffs Digeorge, brutons, SCID, WAS 4 tyoes of immunodeficiencies Digeorge Disease defiicient in T cells Brutons Disease deficient in B cells SCID ( severe combined immunodeficiency) Disease deficient in both T and B cells Immunodeficiency, eczema, thrombocytopenia WAS is characterized by what 3? Leukemoid reaction 3 types of quantiative disorders of leukocytes Leukoerythroblastic reaction Agranulocytosis Leukemoid reaction Type of quantiative disorder of leukocyte that has reactive leukocytosis above 50x10^9/L with neutrophilia and a marked shift to the left Immature neutrophils, nrbcs, teardrop cell 3 preseents in leukoerythroblastic reaction (poikilocytes) and has also possibility of a space- occupying lesion in the bone marrow 0.5x10^9/L In agranulocytosis the nutrophil counts is less than whst value? Infection, inflammation, stress, administration of Types and numberd of circulating neutrrophils can be Colony stimulating fsctors affected by whhat 4? Toxic granulation It is a dark blue black granules in the cytoplasm of neutrophils Positivr Peroxidase result of toxic granulation Primary, azurophilic granules In toxic graanulation. There is increase in acid mucosubstsnfe within what granules of neutrophils? Dohlee bodied It is a cytoplasmic inclusions consisting of remnants of RNA arranged in parallel rows Cytoplasmic vacuolation It is a morphological changes in leukocytes that reflects phagocutosis either self or extrqcellular material EDTA for more than 2 hrs Cytoplasmic vacuolation can csse also by storage of what? Pyknotic nuclei It is the one that indicate imminent cell death Phagosomes In degranulation. Both orimary and secondary genaules are emptied into what Extracellular spaces In degranulation. Secondary granules are also secreted into what? Neutrophils, eosinophil Degranulation is a common finsing in waaht 2 cells? Cytoplasmic swelling It is a morphologic changes in leuckoytes that may ne caused by actual osmotic swelling of the cytoplasm or by increased adhesiom to the glass sliide by stimulated neutriphils Atypical Most common term used in reactive lymphcoytes 3-7 weeks Incubation period for IM B lymphocytes During the incubation period. Thebvirus prefferentially infects what cells? Leading to lymphocytosis Leukemia It is an abnormal, uncontrolled, proliferation and accumulation ofnone or more of the hematopoietic cells Leukemia It is a disease od the blood forming tissues and the bome marrow is always involved True True or false. The profileration cells can infiltrate other organs Fwver, weight loss, increased sweating 3 major sumptoms of leukemia Dueation ofnuntreared disease 3 xlassificatikns of leukemia Number of wbcs present in PB Type of wbc involved Acute leukemia Type of leukemianthat is rapidly progressive that lasts everal days to 6 monts 2-6 months Subacute leukemia lasts for how long? Chronic leukemia Type of leuekemia that mostly 1-2 years and length is cariable depends on the age and atype of cell involed Leukemic leukemia, sub leukemic lueia, aleukemiv 3 types of leuekemia according to wbcs present in PB leukemia >15 x10^9/L How maany wbcs present in leukemic leukemia <15 x10^9/L with immature and abnoemal wbcs How many wbcs present in subleukemic leukemia <15x10^9/L with no immature or abnormal wbcs How manh wbcs present in aleukemic leukemia Acute leukemia, chronic leukemia 2 types of leukemia accoeding to wbc involved Acute leumeia Type of leukemia that has predominance of immature cell types >30% % blast present in PB during acute leukemia >50% % blast present in BM during acute leukemia Chronic leukemia Type of leuekmia that has predominantly mature cell types <10% blasts in BM 10-30% How many % of blasts can be seen in subacute, or chronic transforming into acute Acute leukemia Type of leukemia that onset is sudden and mostly occurs in children under 14 years of age Normo normo Type of anemia present in acute leukemia Low Blasts cells are seen and plts result of acutenleukemia Chemotherapy Cute leukemia is fenerally treated by what Infection/hemorrhage Most common cause of death of persons with acute leukemia Acute leukemia Subacutenleukemia is treated clinically as what Chronic leukemia Type of leukemia thatbhas insidous onset and frequently asymptomatic for a long time 3 ALL is divided into how many types? 7 AML is divided into how many types? Erythrocytes Cells defect in PCV Granulocytes Cells defect in CML Fibroblasts Cells defect in myelofibrosis Platelets Cells defect in essential thrombocythemia Neutrophils Cells defect in CNL Erythrocytosis Polycythemia cera is also known as what? PV, CML, ET, Myekofibrosis 4 typesnof MPNS PCV Type of MPN that is kind of absolute and primary polychythemia Relative, absolute secondary polyxythemia 2 types of polycythemia vera Relative Typenof polycythemia due to dilution Absolute secondary polycythemia Typenofnpolycuthemia due to secondary hypoxic state (appropriate) or to a renal disorders (inappropriate) Hypercellular Bone marrow is what? In PV Tear drop cells It is a poikilocyte that indicates PV is becoming q PPMM (Primary polycythemic Myeloid metaplasia) CML Tyoe if leukemia due to philadelpia chromsome t(9;22) Leukemoid reaction CML must be diffrenetiated from what? Increased LAP result of Leukemoid reaction Decreased LAP result of CML Poor prognosis Patients with negative philadelphia chromosome usually have what? Idiopathic myelofibrosis, agnogenic myeloid metaplasia Myelofibrosis is also known as what 3? (AMM), Myelofibrosis with myeloid metaplasia Myelofibrosis It is an MPN characterized by fibrosis and granulocytic hyperplasia and increase in defective plts that lyse tonsecretion of grnaules Dacrocytes Poikilocyte present in the blod film of myelofboriss, dry tap ET It is an chronic MPN characterized by thrombocytosis >1000 x10^9/L with spontaneoue aggregation and bleeding Platelet aggregation studies It is a test that differentiates ET from Reactive thrombocytosis Myelodysplastic syndromes It is a conditions in which an abnormal pluripotential stem cell population arises in the BM Fatigeu, fever, bleeding Classic triad of of symptoms of MDS Dyserythropoiesis “megaloblastoid type” Hallmark of MDS True True or false. In MDS there may be a left shift CMML (Chronic myelomonocytic leukemia) All MDS has normal/decreased leukocyte count except what? Which is increased CMML It is the only MPN that has monocyted in marrow blood atleast 20% or >1x10^9/L RA/RC and RARS 2 MDS thatt has <1% blast in PB and <5 in BM RAEB, CMML 2 MDS thst has <5 PB blasts and 5-20 in BM RAEBIT The only MDS that has exact 5 PB blasts and 20-30 in BM RARS The only MDs that has >15% ringed siderocyte RA/RC The only MDS that is variable in Ringed siderocyte T cell disease Pariients with these disease tend to have more aggressive disease and porer response to therapy Chronix lymphocytic leukemia, prolymphocytic 3 types of lymphocyte disorders leukemia, hairy cell leukemia B cell Majority of cases of CLL are what origin CLL It is thee least mature among the lymphocyte disorders Smudge cell Cell present in CLL Rai classification Classifcation used in CLL 5 How manybstages rai classification has Stage 0 Stage of rai classification CLL where patients have better prognosis Prolymphocytic leukemia It is the intermediate in maturiry among lymohocytes and have poorer prognosis Prolymphocyte Predominant cell type in prolymphocytic leukemia Hairy cell leukemia Type of lymphocyte disorder that has presence of mononuclear cells with cytoplasmic projections TRAP Test for Hairy cell leukemia that gives positve result B lynphocytes Type of lymphocyte present in hairy cell leukeemia L1 Type of ALL that has small and homogenoud lymphoblasts L1 Type of ALL that hhas scanty cytoplasm and incospicuous nucleoli L1 It is the nost commonntypebof childhood ALL that has the best prognossi L2 Type of ALL that has large and heterogenous lymphoblasts L2 Type of ALL that has abundant basophilic cytoplasm and nuclei clefted with nucleoli present L3 It is the burkitt type of ALL that has 2% of cases of ALL L3 It is the type of ALL that has poor prognosis M1 Type of AML where the predominant cell is poorly diffeentiated myeloblast Decreased LAP result of M1 and M2 M2 Typenof AML that has predominant cells are myeloid precursors that differentiate beyond the promyelocyticnstage Promyelocyte Predominant cell in M3 T (15;17) Chromosome abnormality in M3 M3 Type of AML where the auer rods is most common 20-80% Monocytes percent in M4 M4E Type of M4 with variable numbers of eosinophils Chromosome 16 M4E chromosome abnormality >80% Percent lymphocytes in M5 M5b 2 forms of M5 M5a M5b Ttpenof M5 ATHAT IS DIFFERENTISTED M5a Type of M5 thar is poorlt differentiated M5 Type of AML that is non specific esterase + and stianing inhibited by addition of fluoride Chromosme 1 Abnormal chromosome in M5 Erythroleukemia, erythremic myelosis, di gugielmos M6 3 names syndrome M6 Type of AML that has immature rbcs and rbcs are predominant but myeloblast still PAS Stain for M6 that is psoitve (rbcs) M7 Type of AML that has megakaryocyte malignancy and atypical megakaryocytes Hodgkins, non hodgkins 2 types of lymphomas Hodgkins Type of lymphomas that isnusally localized Non hodgkins Type of lymphoma that is usually widespread Hodgkins Type of lymphoma that is usally central nodes Non hodgkins Type of lymphoma that is usally innvolved peripheral nodes Hodgkins Typenof lymphoma and is contigous/unifocal spread Non hdogkins Type of lymphoma that is non contigious Non hodgkins Type of lymphoma where extranodal disease is common Non hodgkins Type of lymphoma where peripheral blood is involved Hodgkins Type of lymphoma that has abnormal bizzare cells Non hodgkins Type of lymphoma were cell type resembles normal lymphoid cells Hodgkins Typeof lymphoma that has presence of reed sternberg cell neoplastic cell Non hodgkins Type of lymphoma that is mostly b lymphocytes is neoplastic cell Sclerosis Nodular sclerosis that has present what ++ Nodular sclersosi lymphocyte and tumor cell result Lacunar cells Variiant cell in nodular sclerossi ++++ Lymphocyte ressult ofnnodular lymphocyte predominant + Tumor cell result of Nodular lymphocyte predomiant L and H cell Variant cell in nodular lymphxoyte predominanr Lacunar cells It is a tumor cells with cytoplasmic cleating L and H cells It is a cell thatt has multilobatef nuclrus with delicate nuclear membranes Popcorn ceell L & H cell is aka what Reed sternberg cell It is a giant cell with owl eyed appearance 5-6 types How many typpes of leukocytee if light microscopy (romanowskyL procedure used 10 types Howbmany types of leukocytes have if flow cytomerry procedure is used Neutrophils It is the vast majoritt of circulating leukocytes Monocytes Neutrophils share a common progenitor with what? Granulocyte-monocyte progenitor Progenitor of monocytesbandnnuetrophils G-CSF Major CYTokine of neutrophils Stem cell pool, proliferation/mitotic pool, 3 pools of leukcoytes maturation^storage pool HSCs Cell present in stem cell pool CMP to myelocytes Cell present in proliferation/mitotic pool Metamyelocyte, bands, mature neutrophils 3 cells present in maturation/storage pool Myeloblaats It is the first recognizable development stage in the neutrophils 3 typed How many myeoblasts type we have that hard tondifferentiste Promyelocyte Leukocyte maturation stage that has nucleu sround to oval and is often eccentric Promyelocyte Leukoxyte maturation stage that has paranuclear halo or hof that is usually seen in normal thiis. Promyelocyte Hiw many nuclei promyelocyte has with a diffusely basophilic cytoplasm Myelocyte It is the final stage of leukocyte maturation series which mitosis occurs Dawn of neutriphilia Myelocytebis redfered to as what Metamyelocyte It is the leukocyte maturation series that constitute 3% to 20% of nucleated marrow cells and the most abundat cell in BM Band cell Type of leukocyte maturation stage where nuclear indentation ezceeds ½ the diameter of nucleus 2-5 Howw many lobes neutrophils has? Thar conencted by threadlike filaments Circulating pool, marginal pool In PB, neutrophils are divided among what 2? 7 hrs Half life of neutrophils Integrin and spectrin 2 proteins that are significant importance in allowing neutrophils to marginate as well as exit the blood and tenter tissues via diapedesis Phagocytosis Major function of neutrophils Recognitikn and attachment 4 steps of phagocytosis Ingestion Killing and digestion Formations of NETS IL-3, IL-5 , GM-CSF 3 cytikines of eosiniohils IL-5 Most critical cytokine of eosinophils Myelocyte Eosinoohils has same stages as what? Bilobed Lobes of mature eosinophils Eosinophils It is a wbc that contains characteristic refractile, orange red secondary granules 18 hrs Half life of eosinophils Eosinophils It is a wbc that play important roles in immune regulation Eosinophils It is an wbc that capable of acting as antigen presenting cells and promoting the proliferation of effector cells Eosinophils It is the wbc that regulates mast cell function. Through MBP the release of what? Eosinophils It is the wbc that increased in infection by parasitic helminthes and to destroy these is capable of secretion of major basic protein and eosinophilic cationi cprotein as well as production of reactive oxygen sepcies Eosinophils It is an wbc that is hallmark of allergic disorders Basiphils It is the least numerous wbc of all Basophils Granules of this wbc are water soluble and therefore may be dissolved 60 hrs Lifespan of basophils Basophils It is the wbc thst had IgE receptors that can cause degranulation Basophils It iis an wbc capable of releasing large quantities of subtype 2 helper t cell cytokines Basophils It is a wbc that is initiators of the allergic inflammation Mast cell It is the effector od IgE mediated chronic allergic inflammation Mast cells It is a precursors circulate in peripheral blood for a bried period on their way and not considered to be leukocytes Bone marrow and spleen Masr cell progenitors originate from what 2? M-CSF Major cytokine of macrophage and monocyte Monoblasts, promonocytes, monocytes 3 morphologic stages of monocytes Monocytes It is an wbc that has nucleus kidney shaped and also be deeply indentee (horshoe shaped) or folded on itself Blue gray Color of cytoplasm of monocytes Azure dust/ ground glass appearance Fine azure granules of monocytes are referred as what? Classical, intermediate, non classical monocytes 3 subsets of human monocytes 4 monocytes 1 promonocytesbcan produced how many monocytes 16 In increased need. 1 promonocytes produces how many monocytes? Maeginal and circulating Monocytes can be found in the peripheral blood what 2 pool? Type A cell Macrophage of synovial Dendritic cells Macropahge of lymph nodes Monocytes/macrophages 2 wbcs that can be subdivded into innate immunity, adaptive immunity and housekeeping functions T cells, B cells, NK cell 3 types of lymphocytes T and B cell 2 types ofnlymphcoytes responsible for adaptive immunity NK cell 1 type of lymphcoyte responsible for Innate immunity Adaptive imunity Type of immunity that relies on an enormous number of distinct lymphocytes Adaptive immunity Type of immunity where self antigens are ignored and has self tolerance Antibodies, B cells 2 responsible for humoral immunity Cells directly, T and Nk cell 3 responssible for cellular immunity Resting cells Lymphocytes are not end cells. They are whhat? True True or false. Lymphcoytes recirculate from the blood to the tissues and back to the blood Antigen independent phase Both B and T cells can be subdivided into what phase Antiigen dependent phase BM/Thymus 2 orfan that classified as antigen indpenedent phase Secondary/lymphoid organs Organs clasfied as antigen dependent phase Hematogones Immature B cells are also known as what ? Effector B cells It iis an antibkdy producing cells known as plasma cells and plasmacytoid lymphcoytes CD16, 56, 32, 71 4 CD’s of NK cells B lymphocytes It is a lymphcoyte responsible for anti kdy production and antigen presentation to T cells B lymphcoytes It is a lymphocyte that may be necessary for optimal CD4 activation TH1, Th2, Th17 and t reg CD4 effector lymphcoytes are further subdivided into 4? CD4, CD25 t reg 2 types of T regulatory cells NK lymphocyt It is a lymphcoyte that is part of innate immunity and are capable of killing ceetain. Tumor cells and virus infected cells without prior snesitization
----------END-------- -SOURCE: VLADIMIR NOTES (ENHANCED HEMATOLOGY) BY JAY GABRIEL DIESTO, RMT