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Complete Blood Count (CBC) Item Hemoglobin (Hgb) (g/dL) Hematocrit (%) WBC RBC Mean Cell Volume (MCV) Mean Cell Hemoglobin (MCH) Mean Cell Hemoglobin Concentration (MCHC) Red-Cell Distribution Width (RDW) Platelets Normal Range M: 13 - 17 F: 12 - 16 M: 39 - 50% F: 35 - 48% 4,000 - 10,000 M: 4.5 million - 5.9 million F: 3.9 million - 5.3 million 80 - 100 25 - 35 30 - 37% MCV = Hematocrit / RBC Count Abnormal Size = Microcytosis or macrocytosis MCH = Hemoglobin / RBC Count Abnormal Value = Hyperchromic or hypochromic MCHC = MCH / Hematocrit Basically the same as the MCH, except it takes the hematocrit into account. Sort of like a standard deviation; measure of the variability in red-cell size. Below 100,000: Increased bleeding time Below 50,000: Bleeding (ecchymoses, hematoma) on trivial trauma Below 10,000: Spontaneous bleeding, hemorrhage. Comments Absolute concentration of hemoglobin in blood. Percentage of the volume of plasma occupied by cells. Up to 20,000: expected in reactive leukocytosis 50,000-100,000: leukemoid reaction, or CML
11.5 - 15.5% 150,000 - 450,000
MYELOID SERIES: o o o o o o Myeloblast Promyelocyte: Has characteristic azurophilic granules, which contain a Tissue-Factor like substance which, when degranulated (as in AML), can lead to DIC. Myelocyte Metamyelocyte Band Cell: Increased in a reactive leukocytosis with left shift. Mature PMN
HEMOSTASIS: o PLATELET PLUG FORMATION: Mass of platelets form at site of injury. PLATELET ADHESION: Allow platelets to stick to vessel wall. Von Willebrand Factor, on endothelial cell basement membrane, is responsible for platelet adhesion. When it is exposed, platelets will adhere to vessel wall. Glycoprotein Ib/IX is the platelet protein that is a receptor for Von Willebrand Factor. www.brain101.info
ADP.000: Bleeding time is affected.info o o 2 . Collagen: Exposed collagen indicates injury. hereditary hemorrhagic telangiectasia. ibuprofen. FIBRINOLYSIS: Occurs concurrent with the healing process. Partial Thromboplastin consists of: Anionic surface (koalin. It also binds to fibrinogen to facilitate cross-linking. VIII. Fibrin Degradation Products can block this glycoprotein.brain101. Tissue Factor ------> Factor VII ------> Factor X COMMON PATHWAY: X. ITP. XI. ADP Thrombin: Final product of the coagulation pathway causes platelets to stick together. I. preventing platelet aggregation. Plasminogen is converted to Plasmin by two factors: Tissue Plasminogen Activator (tPA) is released by damaged endothelial cells. Kininogen. VII. COAGULATION INTRINSIC PATHWAY: XII. Partial Thromboplastin Time measures its integrity. II. Ehlers-Danlos syndrome Diseases causing thrombocytopenia: chemotherapy. transfusions required. and Factor XII Factor XII ------> Factor XI ------> Factor IX Factor VIII. NORMAL: 5-10 minutes. V. Urokinase BLEEDING TESTS: o BLEEDING TIME: PROCEDURE: Simply measure the amount of time it takes the patient to stop bleeding.000 Von Willebrand Disease. IX. causing crosslinking between platelets and thus allowing for aggregation. enabling them to cross-link.000 . silica) Phospholipid Calcium www. Anionic Surfaces can activate one of three substances in order to start the Intrinsic Pathway: Prekallikrein. renal failure Von Willebrand Disease. Below 50. and Renal Failure all show normal platelet counts.450.o o PLATELET AGGREGATION: Activate platelets so that they stick to each other. Factor X converts Prothrombin (II) ------> Thrombin Factor V. ABNORMAL: Below 100. common pathway starts when Factor X is activated. Several factors cause the platelets to clump together at the site of injury. Below 20. Kallikrein. Thromboxane-A2 (TXA2) activates these receptors. Aspirin. and Ca+2 are required as cofactors. thrombin. Kininogen. ACTIVATED PARTIAL THROMBOPLASTIN TIME (aPTT): Measures the integrity of the Intrinsic and Common Pathways. PROCEDURE: Partial Thromboplastin is added to citrate-anticoagulated plasma. PLATELET COUNT: NORMAL: 150. DIC. amyloidosis. from have two 1mm incisions on forearm. Use filter paper to dop off the blood every 30 seconds. anionic phospholipid. One way or another. and Ca+2 are then required to activate Factor X EXTRINSIC PATHWAY: Tissue Factor. Glycoprotein IIb/IIIa: Platelet receptor which binds to Collagen.000: Bleeding with minor trauma will occur. Thrombin converts Fibrinogen (I) ------> Fibrin Fibrin cross-links platelets and forms cohesive plug. but still have long bleeding times. ABNORMAL: Long bleeding times are seen with any disorder in forming a platelet plug: Diseases affecting the blood vessel wall: vasculitis. scurvy.000: Spontaneous bleeding will occur. TTP Drugs or diseases affecting platelet function: aspirin. anionic phospholipid. Prothrombin Time measures its integrity.
NORMAL: 12-14 second to form a clot in vitro. SUBTYPES: Type I: Partial quantitative deficiency Type II: Qualitative deficiency. FIBRIN DEGRADATION PRODUCTS: They are elevated in Disseminated Intravascular Coagulation (DIC). Type III: Complete absence. the cofactor required to convert Factor X ------> Factor Xa. which impairs clotting. indicating a defect in the Intrinsic Pathway. but normal platelet count. ecchymosis. o 3 www. ABNORMAL: Vitamin-K Deficiency leads to deficiency of factors in the Extrinsic Pathway (Factor VII). GLANZMANN THROMBOASTHENIA: Abnormality in Glycoprotein Complex IIb/IIIa (Platelet receptor). 50-100 second to form clot. recombinant Factor-VIII. SYMPTOMS: Hemarthrosis: Joint and muscle bleeding with minor trauma. You also see atrophy of muscle distal to the joint. SYMPTOMS: petechiae. HEMOPHILIA-A: PATHOGENESIS: X-Linked. in the Intrinsic Pathway. which shows hyperactive fibrinolysis. the final intermediate in the Intrinsic Pathway. Deficiency of Factor-VIII. PATHOGENESIS: Vit-K Deficiency is caused by broad-spectrum antibiotics (due to lost intestinal flora). RENDU-OSLER-WEBER SYNDROME (HEREDITARY HEMORRHAGIC TELANGIECTASIA): Autosomal dominant. VWF is required for initial platelet-plus formation. which is the Von Willebrand receptor on platelets. dysfunctional protein. Will show times of 18-20 seconds. which chronically leads to osteoarthritis. and fat-malabsorption syndromes. so its deficiency results in shorter halflife for Factor VIII. mucosal hemorrhage. May also see prolonged partial thromboplastin time. which is all of the reagents above. VWF is normally a carrier protein for Factor VIII. TREATMENT: Concentrated Factor-VIII transfusions. PROTHROMBIN TIME (PT): Measures the integrity of the Extrinsic and Common Pathways. Epistaxis. plus Tissue Factor. EPIDEMIOLOGY: Rarer than Hemophilia-A SYMPTOMS: Identical to Hemophilia-A LABS: Prolonged Partial Thromboplastin Time (PTT). with recessive inheritance. some cases of Von Willebrand Disease. and in Heparin therapy. Periorbital Ecchymoses: Racoon Eyes are characteristic finding.info . SYMPTOMS: Recurrent spontaneous hemorrhages from trivial trauma. indicating a defect in the Intrinsic Pathway. Worst form. PROCEDURE: Complete thromboplastin is used. HEMOPHILIA-B: X-Linked. Deficiency of Factor IX. or more recently. BERNARD SOULIER SYNDROME (GIANT PLATELET SYNDROME): Deficiency of Glycoprotein Ib/IX. BLEEDING DISORDERS: Congenital and acquired diseases that lead to excessive bleeding or imbalances in hemostasis. LABS: These patients will have abnormal bleeding time. This is only symptomatic in severe cases. Also abnormal in DIC.brain101. ACQUIRED DISORDERS: VITAMIN-K DEFICIENCY: It will lead to a defect in many coagulation factors of the Extrinsic and Common Pathways. malnutrition. and in some cases hemarthrosis and hematomas. o CONGENITAL DISORDERS: VON-WILLEBRAND DISEASE: PATHOGENESIS: Autosomal dominant (generally) deficiency of Von Willebrand Factor. GI bleeds Ecchymoses LABS: Prolonged Partial Thromboplastin Time (PTT).o o NORMAL: 30 second to form a clot in vitro ABNORMAL: Hemophilia = defect in intrinsic pathway. You will see excessive bleeding but will have a normal platelet count.
000 platelets or less -. Macrophages can release tissue factor in circumstances of shock. placental abruption. prolonged Prothrombin time. leading to intravascular activation of clotting cascade. Type M3: The Promyelocytic azurophilic granules contain a tissue-factor like substance that can cause DIC when released. from renal failure. No spontaneous recovery. and 50% from intestinal bacterial flora. The VWF molecule has molecular weight of around 10 million rather than 1 million. but bleeding times are still relatively okay. or caused by several factors: Pregnancy. The disease usually resolves after 6 months without treatment. Sjögren Certain Malignancies PATHOLOGY: Thrombocytopenia. post-partum state Drugs: Cyclosporin. SOURCES: 50% from diet. and elevated fibrin degradation products. Factors V. and I (Fibrinogen): COMMON pathway factors.20. ecchymoses SUBTYPES: ACUTE ITP: Generally found in children. and heparin (to prevent further intravascular coagulation) IDIOPATHIC THROMBOCYTOPENIA PURPURA (ITP): Autoimmune disease against platelets. rickettsial Collagen Vascular Diseases: Lupus. LABS: Level of thrombocytopenia is severe -. fulminant onset of symptoms. treatment is usually required. It can be idiopathic. which sets off the clotting cascade in the bloodstream. thrombocytopenia. Over-activation of the clotting cascade ------> generation of excessive thrombin and plasmin ------> excessive fibrin degradation products. TREATMENT: Try to treat the underlying cause. Occurs in adults. DISSEMINATED INTRAVASCULAR COAGULATION (DIC): PATHOGENESIS: Caused by the pathologic release of pro-coagulant factors into the bloodstream.but the remaining platelets are hyperfunctional.brain101. if that fails. or other diseases. anemia Elevated creatinine and BUN. and sepsis. SYMPTOMS: Petechiae. Complications of pregnancy: Retained dead fetus. Schistocytes (from microangiopathic hemolysis) found on blood smear. LABS: Platelet count is low. usually females. prolonged bleeding time. CLL. Prolonged bleeding times. SYMPTOMS: Insidious onset showing mild to moderate thrombocytopenia. CHRONIC ITP: ITP lasting more than 6 months. CML. Endotoxic infections. and bleeding ------> end-organ infarcts. which are required to chelate calcium in many calcium-dependent clotting factors. blood clotting. Associated with HIV infections. TREATMENT: Usually responds to steroids. PATHOGENESIS: No preceding antigenic exposure. such as SLE. Hemolytic Uremic Syndrome LABS: Prolonged PTT. PATHOGENESIS: Patients are thought to release an abnormally large amount of Von Willebrand Factor (large multimers of it). X. II (Prothrombin). Lots of things can set off the DIC: AML. Beyond that. www. Vitamin K is normally involved in forming gamma-carboxyglutamate residues. SYMPTOMS: Sudden. mitomycin Infection: HIV. give platelets and plasma (to replace clotting factors). shock.info 4 . Vitamin-K dependent factors: Factor VII: EXTRINSIC pathway factor. do splenectomy. PATHOGENESIS: Usually follows a viral infection. THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP): Simultaneous thrombosis. PATHOGENESIS: Anti-platelet auto-antibodies cause the widespread destruction of platelets -----> low platelet count. because the remaining platelets are hyperfunctional.
or neoplasia. PATHOGENESIS: Several things can cause it. but they will have adequate iron stores in bone marrow. similar to TIA's. LABS: Patients will have low serum iron. leading to purpura. Infiltrative Anemia: Anemia resulting when other things infiltrate the bone-marrow. Machine filters out the clots and the extra large Von Willebrand Factor. Rheumatoid Arthritis). TB). Viruses: Parvovirus B-19. which distinguishes it from iron-deficiency anemia. They appear in the standard Wright Stain blood smear. and treatment must be undertaken quickly. chloramphenicol. gold. Often a prominent symptom. www. and may be acute or chronic.brain101. Metastatic Cancer to bone: Lung. HCV. CLASSIFICATIONS: Hypoproliferative: Decreased production of red-cells. PATHOGENESIS: Abnormal or excessive utilization of iron. Often follows a drug-reaction.info o o o 5 . Reticulocyte Index > 2%: Reticulocytes are signs of effective erythropoiesis and redblood cell proliferation. Reticulocytes are decreased or absent because it is hypoproliferative. breast. Renal Failure: Due to lack of erythropoietin. Reticulocyte Index < 2% Hyperproliferative: Increased destruction of red-cells. and prostate cancers often metastasize to bone. which still have some residual RNA elements left in them. PATHOLOGY: Normochromic anemia. benzene. Renal Failure: Due to thrombosis in renal vasculature. HIV-1 PATHOLOGY: Pancytopenia. POLYCHROMASIA: The histological appearance of reticulocytes. Ionizing Radiation Drugs: Chemotherapy. Very common cause of anemia. SYMPTOMS: The disease is deadly if not treated. in compensation. They are all external (environmental) causes. PURE RED CELL APLASIA: Anemia due to isolated depletion of erythroid precursors in the marrow. Extra transfusions are required. HENOCH-SCHONLEIN PURPURA (ALLERGIC PURPURA): Auto-immune attack against vesselwall. Classical pentad of findings: Thrombocytopenia: Spontaneous platelet aggregation ------> low platelet count Microangiopathic Hemolytic Anemia: RBC's are lysed in small vessels by the presence of intravascular fibrin clots. TREATMENT: Plasmapheresis. chronic inflammation (SLE. insecticides. SYMPTOMS: Usually only a moderate anemia. renal failure almost always leads to anemia. Anemia of Chronic Disease: Normocytic. unrelated body systems. due to chronic infections (Histo. Gaucher's Disease APLASTIC ANEMIA: Deficiency or complete failure to produce all blood cells. Fever: It isn't known why. too. This combined with myocardial hypoxia can lead to an MI or CHF. Neurologic Symptoms: Arteriolar thromboses in brain. SECONDARY ANEMIAS: Anemias occurring secondary to other. Light-headedness Decreased renal perfusion ------> increased erythropoietin Increased Cardiac Output and tachycardia. normocytic or macrocytic. The blood-cells that are there retain normal morphology. pushing out normal cellular elements. ANEMIAS: o GENERAL PROPERTIES: SYMPTOMS: Fatigue Pallor resulting form decreased perfusion of skin. normochromic anemia. and not all patients will show it. anti-convulsants.
middle east. SYMPTOMS: Hypochromic. may be found. as in alcoholism Increased metabolic demand. hepatitis) anyway. MEGALOBLASTIC ANEMIA: Macrocytic. PATHOLOGY: Heinz Bodies. alpha-THALASSEMIA: Deficient production of the alpha-globin chain. pregnancy. Low serum iron. Ferritin: Low serum ferritin indicates low body stores of iron. neoplasia. Bone-Marrow will show absence of iron. THALASSEMIAS: Deficient production of hemoglobin. PATHOGENESIS: The nuclear development of the RBC can't keep up with the cytoplasmic growth.info . in Kupffer cells. CHRONIC PRCA: CHRONIC INHERITED PRCA (DIAMOND-BLACKFAN ANEMIA): Quite responsive to steroids. Erythropoiesis is ineffective. PATHOLOGY: Morphologic Abnormalities: Large RBC's with nuclear-cytoplasmic dyssynchrony Tear-Drop Cells Hypersegmented Neutrophils: One of the earliest signs of disease. because this is a hypoproliferative anemia. 5 or 6 lobes. owing to deficient production of hemoglobin. However. precipitated red blood cells. TREATMENT: Removal of thymus may result in clinical remission. a craving to eat clay. ileal resection. SYMPTOMS: General anemia symptoms. Koilonychia: Spoon-shaped nails can be seen. as in pregnancy Malabsorption (as in Sprue) B-12 Deficiency: Autoimmune Gastritis ------> Pernicious Anemia Fish Tapeworm. so there are some acute conditions in which ferritin may be high (inflammation. hyperchromic anemia. Can see pica. Ferritin is a storage-protein found in liver. PATHOLOGY: Microcytic. 6 www. and may be seen in association with thymoma. ileitis. Ovalocytes: The large RBC's tend to have an oval-shape. because of faulty DNA synthesis. Growth. don't rely on ferritin to make the diagnosis of iron-deficiency anemia. lactation. CHRONIC ACQUIRED PRCA: Immunologic etiology. ferritin is an acute-phase protein. Folate Deficiency: Usually dietary.o o o SUBTYPES: ACUTE PRCA: Acute pure red cell aplasia often follows a viral illness. notably Parvovirus B19 infection. Howell-Jolly Bodies: Nuclear fragments seen in Megaloblastic anemia. Target Cells. LABS: Reticulocyte count is low. Therefore. Increased metabolic requirements. can also be seen. due to increased membrane : cytoplasm ratio. hence the Total Iron Binding Capacity (TIBC) is increased with anemia. due to general Over-activation (via erythropoietin) of the bone marrow. IRON-DEFICIENCY ANEMIA: PATHOGENESIS: Several causes. microcytic anemia. Inadequate dietary intake.brain101. Transferrin: These carrier proteins will be unsaturated and available to bind iron. Thrombocytosis: May see increased platelets in chronic anemia. Most common cause = chronic or acute blood loss ------> intracellular fluid goes into vascular space to replace lost fluid ------> relative anemia results. EPIDEMIOLOGY: Mediterranean. hypochromic anemia. Diphyllobothrium Latum Malabsorption: Sprue.
Howell-Jolly Bodies: Nuclear fragments seen in RBC's. The advantage is thought to be that infected RBC's preferentially sickle and are thus taken to the spleen and sequestered. SYMPTOMS: Chronic. SYMPTOMS: Hemolytic jaundice. because it doesn't contain the beta-chain. hyperproliferative anemia. mild hemolytic anemia. plus a variety of other possible problems. It maintains Glutathione in its reduced (active) form. PATHOLOGY: See lots of target cells -. which. HEMOGLOBIN-C DISEASE: Second most common hemoglobinopathy. found in this disease as well as other diseases. Deficiency of this enzyme makes the RBC susceptible to oxidative damage. can lead to Aplastic crisis. Infarctive Crisis: Most common type of crisis. TREATMENT: Transfusion therapy. MEMBRANE-DEFECTS: Defects in RBC shape HEREDITARY SPHEROCYTOSIS: PATHOGENESIS: Deficiency in spectrin. TREATMENT: Splenectomy is usually curative. PATHOLOGY: RBC's can be sequestered in spleen and prematurely removed from circulation. 7 www. leads to spherical shape of RBC's. with homozygous trait. but defects in Hgb structure. SYMPTOMS: SICKLE CRISIS: Accelerated sickling of cells due to low O2-tension.formed by excess hemoglobin-accumulation in center of cell. The cells can be fine for quite some time. and wind up in the spleen or in an end-organ. Excess alpha-chains precipitate in RBC's. Aplastic Crisis: Some bacterial infection can depress erythropoiesis. HEMOGLOBINOPATHIES: Normal levels of hemoglobin. HEREDITARY ELLIPTOCYTOSIS ACANTHOCYTOSIS GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY: PATHOGENESIS: X-Linked Recessive. PATHOGENESIS: Point-mutation of Glu ------> Val at 6th position of beta-globin chain. plus iron chelators to prevent secondary iron overload from the therapy. SICKLE CELL DISEASE: EPIDEMIOLOGY: Endemic to Sub-saharan Africa. End-organ infarcts. death in utero is inevitable. leading to hemolysis. limiting the spread of infection.info . splenomegaly. FAVISM: Susceptibility to eating Fava beans ------> potentially lethal hemolytic anemia. due to heterozygous advantage conferred against Falciparum Malaria. Pigment gallstones may result from the hemolytic jaundice.o o o SYMPTOMS: There are four alpha-genes. Normocytic. as they go through cycles of deoxygenation and reoxygenation. After several cycles they may become deformed. hyperchromic (no central pallor -.delta2). with an increased amount of Hemoglobin A2 (alpha2.for unknown reasons) anemia. since the spleen is responsible for removing the spherocytes. PATHOLOGY: Deoxygenation (low oxygen tension) causes the HbS to polymerize ------> RBC becomes rigid and non-deformable ------> RBC gets stuck in the microvasculature causing microinfarcts. giving bulls-eye appearance. If all four are deleted. PATHOGENESIS: Substitution of Glu ------> Lys at 6th position of beta-chain. G6PD is an enzyme of the Hexose Monophosphate Shunt pathway. HETEROZYGOUS TRAIT: Very mild. SYMPTOMS: Hemolytic anemia brought on by infection or drugs.brain101. beta-THALASSEMIA: Deficient production of the beta-globin chain. PATHOLOGY: Heinz Bodies are bodies of denatured or precipitated hemoglobin. SYMPTOMS: Both excess hemolysis and ineffective erythropoiesis occurs. and severity of disease depends on how many of those genes are deleted. when combined with normal rate of hemolysis.
Can see thrombocytopenia and granulocytopenia. due to repeated infarcts. You can then later determine if it is Warm or Cold autoantibodies. Cells will be destroyed by macrophages in the spleen. TTP. which recognize the Fc portion of IgG. Cardiomegaly. and therefore. HEMOLYTIC ANEMIAS: Hyperproliferative anemias that result from destruction (either mechanical or immunemediated) of RBC's. SECONDARY AUTOIMMUNE HEMOLYTIC ANEMIA: Various causes Drug-induced Hemolytic Anemia: alpha-Methyldopa is the classic drug which leads to autoantibodies and can result in hemolytic anemia (1% incidence). PATHOGENESIS: 50% idiopathic. PATHOLOGY: SCHISTOCYTES are broken-up red blood cells that result from mechanical hemolysis. DIC. but in some downstream function needed to get DAF into the membrane LABS: The hemolysis can affect all cell-types. ischemic attacks. PATHOLOGY: Howell-Jolly Bodies: Nuclear fragments seen in RBC's. SYMPTOMS: Classic symptom is periodic morning red urine. classically found in microangiopathic and macroangiopathic anemias. Alloimmunization of RH negative mother by fetal Rh positive cells may occur at delivery. which can lead to Pigment (Bilirubin) Gallstones. and most others associated with CLL or Lymphocytic Lymphoma. Spherocytes are found in autoimmune hemolytic anemia. it is future pregnancies which are at risk of developing hemolytic disease. or secondary to some cause. in small vessels. with sudden pooling of RBC's and rapid fall in hematocrit.brain101. HUS. Hemolytic Jaundice is frequently seen. MACROANGIOPATHIC HEMOLYSIS: Damage by artifical heart valves. TRAUMATIC HEMOLYTIC ANEMIAS: MICROANGIOPATHIC HEMOLYSIS: RBC's being damaged by intravascular fibrin-clots. as well as anemia. Osteomyelitis from infections and microinfarcts in bone marrow. PATHOLOGY: Usually IgG antibodies directed against the Rh antigen on RBC's. CNS: Strokes. are commonly found with hemoglobinopathies. AUTOIMMUNE HEMOLYTIC ANEMIA: COOMBS TEST: Add Anti-Rabbit IgG to RBC's and see if you get agglutination. neurologic complications. precipitated hemoglobin inside RBC's. Poor splenic function leads to propensity for infections.info 8 . PATHOGENESIS: Can be idiopathic. PATHOLOGY: Polychromasia (high reticulocyte count) indicates effective erythropoiesis. SUBTYPES: Come in the warm and cold flavors: WARM-REACTING HEMOLYTIC ANEMIA: Hemolysis occurs at 37C. The use of RhoGam has greatly reduced the incidence of RH hemolytic disease. ERYTHROBLASTOSIS FETALIS: Hemolytic disease in the fetus or neonate due to maternal antibodies against fetal RBC's. Most common cause of death in early life from Sickle Cell. SPLEEN: Small and fibrotic in chronic disease. www. often found in hemolytic anemias. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH): PATHOGENESIS: Deficiency of functional Decay Accelerating Factor (DAF) on RBC membranes ------> complement is unregulated and disinhibited ------> complement-mediated lysis of RBC's ensues. LDH is elevated. Also quinidine. CV: MI. It is not complement-fixing. CHF. SLE Cancers. especially CLL. Actual genetic defect is not in DAF itself. Hemolytic disease may occur when the maternal antibody is against a fetal antigen of the RH or ABO blood group system. PATHOLOGY: Heinz Bodies.o Sequestration Crisis: Reactive hyperplasia of spleen.
possible left shift). due to inappropriate sequestration of cells in the big spleen.brain101. It is complement-fixing. HYPERSPLENISM: o o PATHOGENESIS: Often caused by portal hypertension or liver disease. o o Refractory Anemia: Unexplained. MYELODYSPLASIA: A group of disorders that are considered to be pre-Leukemic -. HEMOGLOBINS: Hemoglobin-A Hemoglobin-A2 Hemoglobin-F Hemoglobin-S alpha2. anemia. delta2 alpha2. and is sequestered by Kupffer cells in the liver. beta2 alpha2. Sickle crisis can result from low O2-tension. granulocytopenia. Cytotoxic T-Cells CALLA: Common Acute Lymphoblastic Leukemia (ALL) Antigen. beta2 (beta: Glu-6 ---> Val) Hemoglobin-C alpha2. common to all leukocytes. Greater than 30% blasts is diagnostic of AML. Compensatory bone-marrow hyperplasia (polychromasia. directed against the I (blood-type) antigens on RBC's. SYMPTOMS: Triad of findings Splenomegaly Peripheral cytopenia of varying degrees.precursors to acute leukemia. Predominant hemoglobin in adults Found in normal adults Fetal (cord) hemoglobin. normochromic anemia with less than 5% blasts. www. with higher O2-binding affinity Sickle-Cell Hemoglobin. common to all T-Cells. beta2 (beta: Glu-6 ---> Lys) Hemoglobin-C Disease.info 9 . gamma2 alpha2. B-Cell marker Stem-Cell Marker LCA: Leukocyte Common Antigen. T-Cell Helper T-Cell Suppressor. COLD-REACTING HEMOLYTIC ANEMIA: Hemolysis only occurs at cool temps less than 37C PATHOLOGY: Usually IgM antibodies. Selected Cluster Designation (CD) Markers: CD3 CD4 CD8 CD10 CD20 CD34 CD45 T-Cell Receptor (TCR). Refractory Anemia with Excess Blasts (RAEB): Refractory Anemia with 5-20% blasts. normocytic. Can see thrombocytopenia. Second most common hemoglobinopathy.
This is seen especially in burn patients. characteristic of Infectious Mono. Also see Atypical Lymphocytes. Severe pancytopenia.100. PATHOGENESIS: Some people think it is a clonal proliferation. sepsis. to 50. but can affect bones. REACTIVE LEUKOCYTOSIS: Physiologic response to infections. SYMPTOMS: Characteristic triad of Diabetes Insipidus. with suppurative. Bony Lesions. Analogous cells are found all over body. Dohle Bodies: Can also use this finding to rule out CML. Rheumatoid Arthritis AIDS Lymphadenopathy: Generalized lymphadenopathy maybe the presenting symptom in HIV infection. indicating a T-Cell proliferation. found in the skin.000 . but multifocal. Ring Sideroblasts are red-cells with iron-laden mitochondria forming rings around the rim of the nucleus. SUBTYPES: EOSINOPHILIC GRANULOMA (Unifocal): 75% of cases.o PATHOLOGY: Tend to have some immature red-cells in bone-marrow. It looks histologically identical lymphogranuloma venereum (Chlamydia Trachomatis L1-L3). Some etiologies: INFECTIOUS MONONUCLEOSIS: Shows follicular hyperplasia. derived from bone marrow. but can also affect bones. lymphadenopathy. Often affects lungs.brain101. Mildest form. some metamyelocytes). Often affects pituitary gland. but pathogenesis remains uncertain. It must be distinguished from Chronic Myelogenous Leukemia. SYMPTOMS: Pharyngitis. The rings stain with Prussian Blue. and severe infection. PATHOLOGY: Follicular Hyperplasia.000. o o LANGERHANS CELL HISTIOCYTOSIS (HISTIOCYTOSIS X): o o o Langerhans Cells: Tissue macrophages. LEUKEMOID REACTION: A reactive leukocytosis showing a huge increase in the WBC-count. which will show a similar WBC count with similar differential. 10 www. Leukocyte Alkaline Phosphatase activity is high in the leukemoid reaction. particularly of posterior cervical nodes. necrotizing granulomatous inflammation. maybe hepatosplenomegaly. Toxic Granulation: The presence of this characteristic finding in the cells can be used to distinguish the Leukemoid Reaction from CML.info . CHRONIC LYMPHADENITIS: PATHOGENESIS: You can't tell what is causing it by the histology. such as Kupffer Cells of liver and Mesangial Cells of kidney.e. affecting young males. Exophthalmos. NON-NEOPLASTIC WBC ABNORMALITIES: o o AGRANULOCYTOSIS: Bad reaction to certain drugs. showing high WBC count with a left shift (i. DERMATOPATHIC LYMPHADENOPATHY: Reactive changes in lymph nodes secondary to a chronic dermatosis. Non-specific findings. But all three only occur in 15% of cases. excess bands. NEUTROPHILIC DISORDERS: CHRONIC GRANULOMATOUS DISEASE MYELOPEROXIDASE DEFICIENCY CHEDIAK-HIGASHI SYNDROME o BENIGN LYMPHADENOPATHIES: o ACUTE LYMPHADENITIS: CAT-SCRATCH DISEASE: Bartonella Henselae infection. but not in CML. HAND-SCHULLER-CHRISTIAN DISEASE (Multifocal): Same histopathology.
abl codes for Tyrosine Kinase Activity. GRANULOMA: Eosinophils and Langerhans Cells make up the characteristic granulomas. erythropoietin-independent growth of red-cells. t(9:22). Decreased or normal erythropoietin. abl is fused to bcr on Chromosome 22. to suppress proliferation of progenitor cells.000 shows left-shift with myelocyte bulge (too many myelocytes in peripheral blood). CHRONIC MYELOGENOUS LEUKEMIA (CML): PATHOGENESIS: Philadelphia Chromosome is found in all cases of CML.o LETTERER-SIWE DISEASE (Disseminated): Presents with skin rash.increase in all cell-types: erythrocytosis. result from a few different translocations. such that bcr drives the over expression of the abl gene. It usually converts to AML (rapid death in matter of months). Normally peripheral blood should have no myelocytes. sweating. www. leukocytosis. Chronic Phase: Insidious. neutropenia. Median 5-yr survival is 3-4 years. thrombocytopenia. leukopenia. fever. CML shows fewer than 30% blasts. which distinguishes CML from reactive leukocytosis and from the other myeloproliferative disorders. thrombocytosis.brain101. Bone-marrow transplant is improving the outlook of CML. thrombocytopenia. abl:bcr is the most common and classic translocation. where you see a marked left shift. Fatigue. SYMPTOMS: Varying degrees of splenomegaly (extreme in Myeloid Metaplasia) Varying degrees of cytopenia: anemia. It may. Characteristic granules that help to identify Langerhans Histiocytes. Leukocyte Alkaline Phosphatase (LAP): It is absent (low score).info o o 11 . The initial error occurs in a multipotential stem-cell. HEMATOLOGIC NEOPLASMS MYELOPROLIFERATIVE DISORDERS: o GENERAL PROPERTIES: PATHOLOGY: Basophilia is characteristically found in all of the myeloproliferative disorders. Most or all cell-counts are mildly high. Basophilia TREATMENT: Interferon-alfa has been used recently. and the cancer can manifest in all subsequent cell-lineages. but not absent as in CML. Blast Crisis: Leukemic conversion occurs in 70% of cases ------> rapidly progressive anemia. DIAGNOSIS / PATHOLOGY: Leukocytosis: Sustained leukocytosis > 20. PATHOLOGY: Birbeck Granules: Look like tennis-rackets. lymphadenopathy. SYMPTOMS: Splenomegaly. DIAGNOSIS: High hematocrit: Male > 54%. PATHOLOGY: Pancellular hyperplasia -. or it can convert to ALL. All cell types can be seen in the peripheral blood. Hyper activation leads to uncontrolled growth and cancer. This distinguishes it from CML. LAP is variable. Rapidly fatal disease identified in infants. POLYCYTHEMIA VERA: Idiopathic. found in all forms of the disease. and there is no left shift. Female > 51% Pancytosis with normal differential. however. Tear-Drop Cells are characteristically found. or else the diagnosis is changed to AML.
Bone marrow transplantation shows mixed results. Platelet count of 1 . Other chromosomal abnormalities too. DIAGNOSIS: Diagnose by exclusion. Leukemic conversion occurs in 2-5% of patients. Clonal hemopoietic cells proliferate initially. Can also progress to AMM (myelofibrosis). ACUTE MYELOGENOUS LEUKEMIA (AML): o o EPIDEMIOLOGY: Most common leukemia found in adulthood. and they can go into peripheral blood. and leukopenia. Progression: AML progression can occur in 1-3% of cases. Dry Tap: Bone-marrow tap is difficult to do and is almost always dry. Spent Phase: Red cells settle down. Gastric ulcers. Leukoerythroblastic Reaction: The space-occupying myelofibrosis causes immature blood cells to be kicked out into the blood in disproportionate numbers. Polycythemia Vera). PATHOGENESIS: Clonal disorder arising from an aberrant myeloid precursor cell. polyclonal activation of fibroblasts occurs. TREATMENT: No specific treatment.info . PATHOLOGY: Extramedullary Hematopoiesis occurs in liver and spleen. AMM can also occur as a response to the other myeloproliferative disorders (CML. Specifically you see: Promyelocytes (immature granulocyte) Nucleated RBC (immature RBC) SYMPTOMS: Leukocytosis: Especially early on. Later on as the fibrosis becomes really bad. because the bone marrow is fibrotic and non-functional. (PDGF. 12 www. which shows splenomegaly. Radiation Down Syndrome has propensity to lead to AML. IDIOPATHIC THROMBOCYTHEMIA: PATHOGENESIS: Idiopathic increase in Megakaryocytes. causing fibrosis. In response to this. Massive Splenomegaly: The biggest spleens you'll ever see. erythroblasts. and megakaryoblasts. and you can see a post-proliferative reactive myeloid metaplasia (myelofibrosis). due to fibrosis in marrow. 4% of newly diagnosed adult cancers. Possible causes: Myelotoxic agents: Benzene. SYMPTOMS: Median survival 5-8 years (Rubin says 10 years) Patients may have a thrombotic or bleeding tendency. you will see high WBC count. Microcytic hypochromic anemia is common. intermittent claudication. PATHOLOGY: Megakaryocytes show bizarre (malignant) morphologies. Fibroblasts are thought to be responding to growth signals. and chemotherapeutic alkylating agents are most important ones. TREATMENT: Repeated phlebotomy to remove excess red cells. Splenic radiation and splenectomy are often done. Proliferative Phase: Chronic hyperviscosity. which includes myeloblasts. plus iron supplements to replace iron lost from treatments. the WBC may actually be low. leading to increased platelets. They secrete collagen in the bone marrow. It can be a common endpoint to any of the myeloproliferative disorders. monoblasts. This distinguishes Myelofibrosis from Hair Cell Leukemia. Exclude all the other reasons for having high platelets.o o SYMPTOMS: Median survival is 13 years. TGF-beta) sent out by platelets and megakaryocytes. dry tap.3 million or more. AGNOGENIC MYELOID METAPLASIA (AMM) (IDIOPATHIC MYELOFIBROSIS): PATHOGENESIS: Reactive fibrosis in the bone-marrow.brain101. and you are left with essential thrombocythemia. major thrombotic complications.
or common in adolescents (T-Cell immunotype) or in adults.M7 M0 AML: Minimal differentiation. distinguishing it from the myeloid cells (AML). PATHOLOGY: 60% of cases have cytogenetic abnormalities. particularly cervical nodes. with no cytochemical markers. L3 LYMPHOBLASTS: Burkitt's Leukemia. Flow Cytometry can be used to identify the specific type. neutropenia. Found in AML but not ALL. diagnostic of AML Subtype M5. It basically is finding the same anemias. this leads to DIC. M1 . PATHOLOGY: AUER RODS: Structures characteristic of myeloblasts. found in children 6-11 years of age. IMMUNOTYPES: Several different cell-types can yield ALL. Identical histology to Burkitt's Lymphoma. anemia. TDT-Negative: Terminal Deoxynucleotidyl Transferase is not present in myeloid cells. and showing characteristic starry sky appearance. DIAGNOSTIC CRITERIA: Greater than 30% blasts must be present in the bone marrow or peripheral blood. Most common childhood malignancy. thrombocytopenia. M3 AML: ACUTE PROMYELOCYTIC LEUKEMIA (APL) SYMPTOMS: DIC. Metastasis to testes is common in kids. SYMPTOMS: Hepatosplenomegaly Generalized Lymphadenopathy. Clinically most severe type. thrombocytopenia. a very common complication of APL. www. Occurs especially in bones around face and lymph nodes. PATHOGENESIS: The c-myc gene is involved. distinguishing it from the lymphoid cells (ALL). which changes how the disease would be treated. SUBTYPES: FAB divides it into 8 Subtypes: M0. It is treated with retinoic acid. CLINICAL: Poor prognosis. M5 AML: Monocytic Leukemia. Promyelocytes will show azurophilic granules and Auer rods. which is the diagnostic stain. Larger cells with vacuoles in cytoplasm. GRANULOCYTIC SARCOMA (CHLOROMA): Discrete tumor masses infiltrated into soft tissues. When degranulated. TDT-Positive: Terminal Deoxynucleotidyl Transferase is present in the lymphoid cells. PAS-Positive: Lymphoblasts in general stain positive for PAS. May have CNS involvement. PATHOGENESIS: t(15:17) is the characteristic translocation. Hyperdiploidy is a common abnormality and is a favorable prognostic indicator. B-Cell ALL: More common. L2 LYMPHOBLASTS: Contain prominent nucleoli. which contain a Tissue-Factor-like substance. Subtypes are Pre-Pre-B-ALL. ACUTE LYMPHOBLASTIC LEUKEMIA (ALL): o o o EPIDEMIOLOGY: Childhood. Stains positive (red) with Chloroacetate Esterase. Pre-B-ALL. but with the greater than 30% blasts needed to make a diagnosis. Myeloperoxidase-negative: Only granulocytes have myeloperoxidase. worst prognosis. with greater than 30% blasts are present in the bone marrow or blood. different than other subtypes. DIAGNOSIS: Leukocytosis. M2 AML: AML Without Maturation. plain cells. All the myeloblasts encroach on normal bone marrow function. SYMPTOMS: Granulocytopenia.o o o o Myelodysplastic Disorders (Refractory Anemias) are considered to be pre-leukemic.info o o o o 13 .brain101. common in the children 3-7 age group. as in Burkitt Lymphoma. Null Cell ALL SUBTYPES: 3 Subtypes proposed by the French-American-British (FAB) Group: L1 LYMPHOBLASTS: Small. and Mature BALL (worst prognosis) T-Cell ALL: More common in adolescents. 3500 new cases diagnosed in 1995 in USA. better prognosis. CLINICAL: Found in infants younger than 1. Non-Specific Esterase is a stain that is specific for monocytes. Most common subtype. CLINICAL: Best prognosis. Normocytic normochromic anemia.
It is the most common leukemia in Western countries. and not enough CD4 cells.15. PATHOGENESIS: CLL is a clonal proliferation of immunologically incompetent small lymphocytes. or older than 10 (L3 subtype) Black Male Prominent Race Sex Organ Involvement Cytogenetic Abnormalities White Female Minimal Hyperdiploidy CHRONIC LYMPHOCYTIC LEUKEMIA (CLL): o o EPIDEMIOLOGY: The most common hematologic cancer in the United States. and may infiltrate the liver.000 Children less than 1 (L2 subtype). Diffuse infiltrate of hairy cells into bone marrow and spleen. Lymphocytes between 5. Pan-T-Cell Marker CD5 is expressed. SYMPTOMS: Indolent course.brain101. occurs in the older population and has an indolent course. Tartrate-Resistant Acid Phosphatase (TRAP): Hairy-Cells are TRAP-Positive. due to too many CD8 Suppressor cells. Smudge-Cells: Cell appearance characteristic of CLL. spleen and lymph nodes as well as other organs. SUBTYPES: B-Cell CLL is by far (~95%) the most type of CLL in the USA. which indicates that it is in immature B-Cell (but still not a blast). Immunodeficiency: Results from: Hypogammaglobulinemia and granulocytopenia ------> pyogenic infections. www. This distinguishes the cells from neutrophils which are TRAP-negative.000 is diagnostic. PATHOLOGY: Low-grade B-Cell leukemia. mean survival of 6 years. which are almost always of B-cell phenotype. PATHOLOGY: Lymphocytosis: High lymphocyte-count diagnostic of CLL Lymphocytes above 15. o o EPIDEMIOLOGY: The mean age is 50-60 years old. with an absolute lymphocyte count above 5000 /l. Impaired cellular immunity. Thrombocytopenia Coombs-positive hemolytic anemia PROGNOSIS: Bad prognostic indicators include: Diffuse. as opposed to interstitial or nodular histological patterns in the bone marrow.000 Children 3-7 (will probably be L1 subtype) Greater than 50. CLL involves the bone marrow and peripheral blood. with a mean survival of 6 years.o PROGNOSTIC FACTORS: Favorable Prognosis L1 B-Cell (Pre-Pre-B-All. Found in old folks. Hairy Cells: Cytoplasmic projections on cell-surface of B-Cells.000 is diagnostic. and especially L3 T-Cell ALL Mature B-Cell ALL Clinical Features FAB Subtype Immunotype WBC Count at Diagnosis Age Less than 10.info 14 . if monoclonality is present.000 . o o o o HAIRY-CELL LEUKEMIA: A rare chronic lymphocytic leukemia of B-Cells. with a male predominance. The presence of chromosomal abnormalities. Pre-B-All) Unfavorable Prognosis L2.
General symptoms of a malignancy. indicating an abundance of a single type of antibody. Only rarely do the tumors disseminate through peripheral blood.o o SYMPTOMS: Presenting Symptoms: Classic symptoms. since the origin of the amyloid is directly related to the increase in immunoglobulins. Monoclonal Gammopathy of Unknown Significance (MGUS) is part of the differential diagnosis. Massive Splenomegaly: Due to infiltration of hairy cells into spleen Pancytopenia Dry Tap: Bone-marrow tap is dry. due to reactive fibrosis in the bone marrow. Amyloidosis of Multiple Myeloma is considered to be a primary amyloidosis. It is diagnosed when a monoclonal spike is found in the absence of the other diagnostic features. DIAGNOSIS: Sheets of plasma cells found in the bone marrow. PATHOLOGY: Monoclonal spikes in the Protein Electrophoresis are found. ChediakHigashi) Hodgkin's Disease (post-treatment. fever. found in a middle-aged man. SCID. Single lytic lesion of bone. ROULEAUX: Paraproteins from the monoclonal antibodies tend to make red-blood cells stick together. but aggressive in 15% of cases. Amyloidosis is characteristic of Multiple Myeloma. o PATHOGENESIS: Several causative factors Genetic predisposition Chronic antigenic stimulation: chronic stimulation of B-Cells can cause plasma cell malignancy. No lymphadenopathy Prognosis: Chronic and indolent course.brain101. MULTIPLE MYELOMA: 90% of plasma cell cancers are Multiple Myeloma. night sweats. MGUS later advances to full-blown Multiple Myeloma in many cases. Radiologic demonstration of lytic bone lesions. giving the characteristic "roll-of-coins" appearance. Interferon alfa also used. Bence-Jones Proteins: Immunoglobulin light-chains present in the urine. resulting from chemotherapy) www. causing mesangioproliferative glomerulonephritis and tubulointerstitial inflammation. to inhibit proliferation of hairy cells. Death usually by infection. Up to 20% of cases progress to Multiple Myeloma. Light-Chain Cast Nephropathy: Severe renal involvement. o o o o NON-HODGKIN LYMPHOMAS: o o o STAGING: Progression of tumor is generally: Start in lymph node ------> spleen ------> liver ------> bone marrow -----> peripheral blood. Chromosomal abnormalities OTHER PLASMA CELL CANCERS: EXTRAMEDULLARY PLASMACYTOMA: 5% of cancers. Ataxia Telangiectasia. SYMPTOMS: Painless lymphadenopathy. fatigue. Solitary Osseus Myeloma: 5% of cancers. when they are prominent they are almost always associated with a worse prognosis.info 15 . Plasma-cell cancer usually occurring in upper respiratory tract. TREATMENT: 2-CDA: 2-Deoxycoformycin is a recent treatment that has greatly improved the survival in these patients. Russell Bodies: Eosinophilic cytoplasmic inclusions of excess immunoglobulin. splenomegaly "B" SYMPTOMS: Weight loss. or diffuse demineralization of bone. SYMPTOMS: Lytic Bone Lesions: Characteristic lesions due to cancer cells secreting an osteoclast-activating factor. PATHOGENESIS: Several disorders are associated with increased risk of Non-Hodgkin Lymphoma Sjögren Syndrome HIV: Particularly Burkitt Lymphoma Congenital immune deficiency syndromes (Wiskott-Aldrich. Significant Monoclonal M-Component found in serum or urine. Dutcher Bodies: Eosinophilic nuclear inclusions of excess immunoglobulin.
terminal ileum. PATHOLOGY: Nodular (Follicular): Mostly low-grade. slow growth. carrying a worse prognosis. yet they are still fairly aggressive. based on the rate of growth and aggressivity of the tumor Low-Grade: Proliferative index < 5%. Can be widespread at time od diagnosis. DIFFUSE LARGE CELL LYMPHOMA: Relatively common tumor. Diffuse: Mostly high grade. the more aggressive is the tumor. Sjögren's). but easier to treat by chemotherapy. as in Burkitt Lymphoma. due to reduced Factor VIII (which is mopped up by all the extra paraprotein). HIGH GRADE LYMPHOMAS: o LARGE-CELL IMMUNOBLASTIC LYMPHOMA: PATHOGENESIS: Can be associated with auto-immune disorders (RA. Sometimes grouped with the High-Grade Immunoblastic Lymphoma. paresis. high grades. www. It is caused by high molecular weight paraprotein in the blood. Histological pattern carrier a better prognosis. Aggressive course. Tough to treat by chemotherapy because it's slowgrowing. all others are low-grade. bone marrow. PATHOLOGY: Both CLEAVED and NON-CLEAVED cells are present. hence prognosis ain't so good. Mean survival = 4-5 years MALT LYMPHOMA (MALTOMA): FOLLICULAR (CENTER-CELL) LYMPHOMAS: Follicular (as opposed to diffuse) generally indicates high level of differentiation.secretion of monoclonal IgM. headache. SYMPTOMS: Hyperviscosity Syndrome is seen secondary to the monoclonal gammopathy: Peripheral neuropathy. intermediate. thyroid. the larger the cells. often much greater than 10%. Found in 90% of cases. Follicular Large Cell Lymphoma is the only follicular tumor placed in the intermediate grade category. and a low-grade. Intermediate-Grade: Proliferative Index 5-10% High-Grade: Proliferative Index > 10%. or other immune disorders. such as stomach. It may be classified with the other follicular lymphomas in future. deafness. Extranodal Sites: Tumor usually presents at an extranodal site. WALDENSTROM MACROGLOBULINEMIA: SLL with Plasmacytoid Differentiation most often presents with a monoclonal gammopathy -. AIDS patients often get this lymphoma. LOW-GRADE LYMPHOMAS: o SMALL LYMPHOCYTIC LYMPHOMA (SLL): Equivalent to Chronic Lymphocytic Leukemia (CLL) SYMPTOMS: Low-grade. SUBTYPES: FOLLICULAR SMALL-CLEAVED CELL LYMPHOMA: FOLLICULAR MIXED SMALL CLEAVED AND LARGE-CELL LYMPHOMA: o o INTERMEDIATE-GRADE LYMPHOMAS: o o FOLLICULAR LARGE-CELL LYMPHOMA: This is the only subtype of follicular lymphoma associated with an aggressive clinical course (hence it is intermediate grade). This results in inhibition of apoptosis ------> uncontrolled growth. Indolent course. PATHOLOGY: Generally. One third will see dissemination to blood ------> clinical picture identical to CLL. PATHOGENESIS: t(14:18) translocation results in over-expression of bcl-2 oncogene. indolent lymphoma. Tendency to bleed.brain101. coma. SYMPTOMS: They are intermediate grade.info 16 .o o International Working Formulation: Classifies lymphomas into low.
PATHOGENESIS: c-myc: Translocation t(8. Sézary Cells in blood have a perinuclear ring of PAS-positive vacuoles. Only 15% EBV. Starry sky: Characteristic appearance of Burkitt's. EBV: EBV infection occurs in 80% of endemic Burkitt lymphomas. Sporadic Burkitt Lymphoma: USA. endemic to Carribean and West Africa.brain101. Epidermotropism: T-Cells migrate up into the epidermis. This results in a dominant monoclonal colony of B-Cells. Type L3 (Burkitt's Leukemia). secondary to leukemic dissemination of the cancer. SÉZARY SYNDROME: Generalized erythroderma (intense and widespread reddening of skin). Burkitt-Like Lymphoma: Rare MISCELLANEOUS LYMPHOMAS: o CUTANEOUS T-CELL LYMPHOMA (MYCOSIS FUNGOIDES): Primary lymphoma of the skin. can usually be diagnosed at this stage. in late-stage disease. analogous to Acute Lymphocytic Leukemia (ALL). DIFFUSE SMALL NON-CLEAVED CELL LYMPHOMA (BURKITT LYMPHOMA): Extremely fastgrowing B-Cell Lymphoma. in children in Africa.14) is present in 80% of cases. Most common on face and in body folds. HIV: Burkitt Lymphoma is the most common lymphoma seen in AIDS population. STAGES of Disease: Patch Stage: Benign chromic dermatoses. Disease is widespread and prognosis is poor. Late spread shows involvement of CNS and leptomeninges. SYMPTOMS: Often presents as big mass in mediastinum.info . Often present with abdominal masses rather than mass in jaw. Often spreads to bone marrow and disseminates as Leukemia. ADULT T-CELL LEUKEMIA / LYMPHOMA: Caused by the HTLV retrovirus. SYMPTOMS: Has a chronic. TDT-Positive. Lymphadenopathy presents as mass in jaw. indolent course. 80% EBV. Sea of blasts. c-myc is activated by its proximity to the heavy-chain gene. which are selected for based on the mutation. PATHOLOGY: Usually T-Cell lineage. Pautrier Microabscesses: Characteristic microabscesses of cancer cells. Pruritus and recurrent cutaneous infections are the most common chronic symptoms. PATHOGENESIS: PATHOLOGY: T-Cell origin. Overall poor prognosis. Plaque Stage: Well demarcated plaque. frequently ulcerate. as the cells are of lymphocytic lineage. throughout the tumor. Tumor Stage: Microabscesses and epidermotropism occur. in children in Africa. which distinguishes this tumor from most others. EPIDEMIOLOGY: Adolescent and young-adult males. immature cells.o o PATHOLOGY: Immunoblasts are identified by huge nucleoli in the center of the cell. LYMPHOBLASTIC LYMPHOMA: Analogous tumor to Acute Lymphocytic Leukemia. Cannot yet be diagnosed as Mycosis Fungoides. o 17 www. suggesting origin the thymus. found in epidermis. This appearance results from tingible-body macrophages. PATHOLOGY: Burkitt Lymphoma is the fastest growing tumor known to mankind. which swallow up dead tumor cells. SUBTYPES: Endemic Burkitt Lymphoma: Africa.
> 50: Generally get the Mixed Cellularity subtype. the more background (non-malignant) lymphocytes you have in the tumor. Stage-II: Two or more sites. Bimodal Age Distribution: 15-35. Stage at diagnosis correlates with prognosis.info Hodgkin's Disease Localized lymphadenopathy Always an orderly. Stage-I: Single node.EQUIVALENT NEOPLASMS: Leukemia Chronic Lymphocytic Leukemia (CLL) Lymphoma (Low-Grade) Small Lymphocytic Lymphoma (SLL) Acute Lymphoblastic Leukemia (ALL) (High-Grade) Lymphoblastic Lymphoma Acute Lymphoblastic Leukemia (ALL). than older than 50. Ann-Arbor Staging System: Stages I-IV. the better the prognosis. B-Symptoms: The presence of fever. www. Waldeyer's Ring Uncommon Non-Hodgkin's Lymphoma Generalized Lymphadenopathy Spreads everywhere unpredictably Predominantly Waldeyer's Ring Common (30% of cases) o o 18 . or localized contiguous involvement. Resembles owl's eyes. Stage-III: Both sides of diaphragm. Reed-Sternberg Cells are the only malignant cells. contiguous spread Mesenteric nodes. 15-35: Generally get the Nodular Sclerosis subtype.brain101.NON-HODGKIN Clinical Feature Lymphadenopathy Spread of Tumor Involved nodes Extranodal Involvement HODGKIN'S LYMPHOMA: o EPIDEMIOLOGY: Relatively rare disease. involving spleen. with a good prognosis. PROGNOSIS: Histologic Subtype: Generally. night sweats. REED-STERNBERG CELL: Large. PATHOLOGY: The entire disease is based on the histopathology. All other cells express disease only in their reaction to Reed-Sternberg cells. correlates with a bad prognosis. with a worse prognosis. the presence of characteristic Reed-Sternberg cells. Stage-IV: Disseminated. used to stage Hodgkin's Disease. but origin is still uncertain. binucleated cell with giant eosinophilic inclusions. Type L3 = Burkitt Leukemia (High-Grade) Diffuse Small Non-Cleaved Lymphoma = Burkitt Lymphoma HODGKIN'S -vs. Latest research says it probably is a B-Cell lineage. on the same side of the diaphragm. or single primary site.
and Non-Hodgkin's Lymphoma. Often presents with mediastinal lymphadenopathy. Anergy to skin tests is often noted at diagnosis. They're not sure which comes first -. Immunodeficiency: Usually presents at time of diagnosis. which distinguishes it from other forms of Hodgkins.info . Poorest prognosis. This may later by grouped with Non-Hodgkin's B-Cell Lymphomas. because of lots of non-malignant lymphocytes and few ReedSternberg cells. PATHOLOGY: LACUNAR CELL is a unique form of Reed-Sternberg cell. Can also present with standard cervical lymphadenopathy. SYMPTOMS: Lymphadenopathy. MIXED CELLULARITY: Found in 40's to 50's. or as time progresses. intermediate prognosis. SYMPTOMS: Usually found in young women.o o o SUBTYPES: LYMPHOCYTE-PREDOMINANT: Least common subtype. But. secondary malignancies subsequent to the chemotherapy are especially high with Hodgkin's Disease.brain101. pathognomonic for this subtype of Hodgkin's Disease.the disease or the immune deficiency. B-Symptoms correlate with poor prognosis and are present in 40%. NODULAR SCLEROSIS: Good prognosis. good prognosis. Common Secondary Tumors: AML. CLINICAL: Excellent prognosis. 19 www. LYMPHOCYTE DEPLETED: Found in elderly men. TREATMENT: Chemotherapy is usually effective.