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19 July 2010 Hematologic Disorders Maria Santa Portillo


Caring for Clients with Hematologic Alterations

makes 7-10% of the total body weight female: 4 5 liters male: 5 6 liters pH: 7.35 7.45 specific gravity: 1.048 1.066 FUNCTIONS: link to the different organ system transporter/carrier oxygen and nutrients, CO2, glucose controller if you lose blood homeostasis defender to foreign activities BLOOD COMPONENTS:

biconcave disk allow RBC to pass in small vessels easily deformed in any shape lifespan = 120 days Hematopoeisis: Production of RBCs in utero: liver, spleen, lymph nodes in adult: bone marrow (sternum, ribs, vertebrae, pelvis)
yolk sac

liver, spleen, and lymph nodes

bone marrow

RBC differentiation:
pluripotent stem cell proerythroblast (nucleated) basophil erythroblast reticulocyte (young RBC) mature erythrocyte

PLASMA liquid part of blood yellow in color 90-91% water, 6-8% proteins, 2% other solutes PROTEINS Albumins prevents colloidal osmotic pressure big protein molecules remains in the interstitial space regulates blood volume preventing the occurrence of edema prevents the shifting of water Serum Globulins Alpha, Beta, Gamma for clotting factors Fibrinogen, Prothrombin, Plasminogen for clotting factors CELLULAR COMPONENTS ERTHROCYTES (RBC) roles (BCC) Buffering power Carry Hmg (hemoglobin) most important Hmg has carbonic anhydrase (enzyme that converts CO2 and H2O to hydrogen and carbonic acid H2 + CO3) Catalyzes

reticulocytes = body is attempting to compensate RBC Regulation: Tissue oxygenation (PPALL) Poor blood flow Pulmonary disease Anemia Low Hmg Low blood volume Erythropoietin 90% kidney 10% liver * common endpoint = hypoxia ( O 2 levels in tissues)
O2 levels; hypoxemia in blood hypoxia; O2 in the tissues kidneys secrete erythropoietin hormone acts on bone marrow (stimulated) facilitate production of RBCs

RBC Maturation:

Folic Acid sources: green leafy vegetables Vitamin B12 sources: meat ONLY

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2 important vitamins: Folic acid and Vit B12

required as an essential building block of DNA of RBCs

nuclear maturation and division

Formation of Hemoglobin:
protoporphorin IX + Fe++ heme formation heme + globin hemoglobin chain 2 alpha chains + 2 beta chains Hemoglobin A (HgA) - adult Hmg

Non-Granular/Mononuclear Lymphocytes B lymphocytes humoral immunity T lymphocytes thymus, cellular immunity Monocytes young macrophages macrophages engulfs PLATELETS: part of a bigger cell/fragment characteristics: tiny cell fragments no nucleus formed in bone marrow 150,000 300,000 uL lifespan: approximately 10 days function plays a role in blood coagulation **We need 100B new blood cells a day Mechanism of Hemostasis: orderly, stepwise process for stopping bleeding that involves: Vascular phase: vasoconstriction of blood vessels > diameter of blood vessel > blood volume Platelet plug formation: aggregation of platelets > collagen stimulates platelets > seal off injury Coagulation: extrinsic and intrinsic factors Fibrinolysis or Clot dissolution
vasoconstriction (exposure of collagen) platelet formation (intrinsic L pathway) coagulation clotting factor

Iron Cycle:

absorbs and stored in the duodenum forms of iron in body 4 5grams Fe 65% Hmg 15 30% ferritin (stored iron in liver) 4% myoglobin (muscles) 19% variations heme compounds needed for intracellular oxidation 0.1% transferrin after RBC death, Fe is being phagocytized by the spleen Fe is being reused

extrinsic mechanism (tissue thromboplastin)

intrinsic mechanism (blood vessel injury)

LEUKOCYTES (WBC) fighting for infections mobile units of bodys protective system formed in: bone marrow and lymph tissues function: seek out and destroy types: Granular (BEN)
NORMAL VALUES FUNCTION/FEATURES IgE receptors (during inflammatory response) Histamine Heparin life span = 8 12 days E(a)limentary and respiratory tract E(a)llergy Parasitic infection *asthma = eosinophils first line of defense against bacterial infection neutrophils = infection

prothrombin (common pathway) thrombin fibrinogen > fibrin (clot) activation of clot plasminogen > plasmin


0 0.7%


in Hmg content or red cell mass impairs oxygen transport CLASSIFICATIONS: based on morphology (cell size and shape) normocytic (normal) microcytic (small) macrocytic (big)


0.5 1%


55 70%

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ETIOLOGY: inadequate RBC production premature or excessive RBC production blood loss (hemorrhage) deficits in nutrients (iron, Vit B12) hereditary factors (sickle) chronic diseases (production of erythropoietin TYPES: nutritional anemia (inadequate intake) Iron-deficiency (IDA) Vitamin B12 deficiency Folic Acid deficiency anemia hemolytic anemia (characteristics) Sickle cell anemia Thalasemia Glucose-6-phosphate dehydrogenase bone marrow depression anemia Aplastic anemia IRON DEFICIENCY ANEMIA NO HMG FORMATION BELOW NORMAL TOTAL BODY IRON INADEQUATE HMG PRODUCTION FOR BODY REQUIREMENTS ETIOLOGY: (5 IS) Increase blood loss Insufficient dietary Fe intake (doesnt eat green veggies) Impaired GIT absorption Increased Fe requirements (menstruation, pregnancy) female: 2mg iron intake additional Infection LAB FINDINGS: microcytic, hypochromic RBC chromic something is wrong with the Hmg content Hmg lower than hct (hematocrit) serum Fe concentration total Fe binding capacity serum ferritin (because no iron) reticulocyte count (because no iron that can produce) ASSESSMENT FINDINGS: (FE KAP) Fatigue low O2 capacity in tissues (common) Exercise Tolerance is decreased Koilonychia nails become brittle and spoon-shaped (d/t insufficient supply in the periphery) Angular cheilitis mouth ulcerations, lesions in oral mucosa Pica, Pallor pica feeling of deficiency of something so you crave (ice, flour, not common) pallor pale GOALS OF MANAGEMENT: correction of underlying cause treatment through diet and supplemental iron preparations Vitamin C

SUPPORTIVE MANAGEMENT FOR PATIENTS WITH ANEMIA: meet patient nutritional needs small frequent meals oral lesions soft, cool, bland foods dyspepsia eliminate spicy foods, milk and dairy products anorexic and irritable give preferred food, accompany during meals fatigue adequate rest periods proper scheduling of activities prepare patient for diagnostic testing schedule all tests to avoid disrupting patient meal, sleep and visiting hours finish first light activities before doing heavy ones prevent complications observe for signs of bleeding monitor transfusions warn patient to change position slowly to minimize dizziness induced by cerebral hypoxia set limitations on acts assess level of tolerance on acts frequent rest periods pace activities NURSING RESPONSIBILITIES IN FE PREPARATION: assess for use of drugs that can interact with Fe administer with orange juice administer 1 hour AC or 2 hours PC AC before meals PC after meals do not give with milk and antacids give with straw for liquid preparations monitor for Fe toxicity loss of appetite, fatigue, wt loss, HA, bronze/gray hue to the skin, dizziness, nausea, SOB monitor Hmg and reticulocyte count stools may be dark green fluids and fibers to prevent constipation INJECTING IRON SOLUTIONS: use of z-track technique in the buttocks to prevent staining skin apply pressure, dont massage watch out for dizziness, HA, thrombophlebitis in IV site regular check-up and blood studies pinch and twist


MACROCYTIC ANEMIA TYPES: Vitamin B12 deficiency (Pernicious) anemia Folic Acid deficiency anemia

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FOLIC ACID DEFICIENCY ANEMIA CAUSES: (ABCDEF) Alcohol abuse Bacteria Cooking excessive Diet, Drug therapy (anticonvulsants) E(i)nfants (limited storage capacity) Folic acid requirement increased FOLIC ACID ADMINISTRATION: assess the use of other drugs do not mix with other drugs in the same syringe (if IV form) monitor for hypersensitivity urine may become darker yellow excess alcohol intake may folic acid requirements PERNICIOUS ANEMIA CAUSES: (VAGGGO) Vitamin B-12 deficient in diet (no meat) Autoimmune reaction body attacking on B12 (intrinsic factor) > to be absorbed in ileum for RBC production Alcoholics Gastric atrophy Gastrectomy (no IFintrinsic factor) Gastritis, chronic Older individuals ASSESMENT FINDINGS: beefy red tongue (positive for PA) general s/sx of anemia weakness smooth sore tongue diarrhea paresthesia abnormal sensation tingling sensation in the fingertips impaired coordination and position sense confusion and behavioral change LAB FINDINGS: serum folate levels serum Vit B12 Schilling test measure of excretion of orally administered radionuclide labeled B12 urine samples are collected Stage 1: Patient is given radioalabeled Vit B12 to drink or to eat IM is given at around the same time (temporary saturate B12 receptors in liver w/o normal B12) Normal result at least 5% radiolabeled Vit B12 in urine over first 24 hours Pernicious anemia less than 5% radiolabeled Vit B12 Stage 2: if abnormal, test is repeated with additional oral IF Normal urine collection = lack IF or pernicious anemia

low result = abnormal intestinal absorption

PART 1 TEST RESULT Normal Low Low PART 2 TEST RESULT Normal Low DIAGNOSIS Normal/Vit B12 deficiency Pernicious Anemia Malabsorption

Result: no trace in urine = (+) pernicious anemia to absorb Vit B12, give IF after giving IF > trace seen > (+) NURSING MANAGEMENT: Parenteral replacement of Vit B12 Diet VITAMIN B12 ADMINISTRATION: Cyanocobalamine (oral, IV, IM) do not expose crystalline injection to light assess for other drugs that might interfere with effectiveness administer parenteral doses IM or deep SQ to local irritation a burning sensation with injection is temporary no to alcohols monitor Hmg, RBC, reticulocyte count, and K levels medication is taken for life SPECIAL CONSIDERATIONS: monitor pulse rate heart pumps and pumps compliance with lifelong Vit B12 diet plan activities (d/t fatigue) good oral hygiene (d/t angular cheilitis) CNS complications HEMOLYTIC ANEMIA SICKLE CELL ANEMIA ABNORMAL SHAPE OF RBCS (CRESCENT SHAPE) SEVERE, CHRONIC, INCURABLE, HEMOLYTIC ANEMIA RESULTING


NC + C = 0% chance C + C = 25% chance with disease + C = 50% chance

SICKLING PROCESS: Triggers Stress (anxiety, etc) Infection Cold environment Physical exercise Acidosis, Altitudes (high) Dehydration

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exposure to low O2 defective Hmg sickle cells fragile and sticky impede circulation microinfarcts tissue hypoxia

alert young women with sickle cell anemia that pregnancy carries a highrisk for them explain complications and how to prevent crises APLASTIC ANEMIA PRIMARY CONDITION OF BONE MARROW STEM CELLS BONE MARROW FAILS TO PRODUCE ALL THREE TYPES OF BLOOD CELLS (PANCYTOPENIA ALL BLOOD COMPONENTS ARE LOW LEVES) anemia no RBC infection no WBC bleeding no platelet ETIOLOGY: 50% idiopathic chemical damage: benzene, arsenic, chloramphenical, chemo drugs viral infections: mononucleosis, Hep C, HIV exposure to radiation CLINICAL MANIFESTATIONS: anemia leukopenia low levels, recurrent/multiple infection thrombocytopenia petechiae, tendency to bleed excessively MANAGEMENT: eliminate any identifiable cause explain patient condition educate public about hazards of toxic agents prevent infection prevent bleeding prevent falls continue normal lifestyle with appropriate restrictions BONE MARROW TRANSPLANT: 3 categories Autologous from patient Syngeneic transplant taken from identical twin Allogeneic transplant from an HLA-matched sibling or an unrelated HLA-matching donor HLA = human leukocyte antigen; match required to prevent rejection Steps Conditioning pre-treatment of bone marrow transplant recipient with high doses of chemotherapy +/total body irradiation to destroy the malignancy Bone Marrow Harvesting needle is inserted into pelvis bone marrow is collected from the donor under general anesthetic, 5001200ml of bone marrow from pelvis Processing removal of red cells and to concentrate the mononuclear cells

SICKLE CELL CRISIS occurs when client experiences O2 resulting in the enlargement of rigid sickle-shaped cells vaso-occlusive crises/pain crises aplastic crises infection with human parvovirus hyperhemolytic crises sequestration crises other organ pool the sickled cells DIAGNOSTICS: sickle slide preparation (microscopic) sickle-turbidity tube test Hb electrophoresis diagnostic test x-ray abdominal UTZ trancranial dopper UTZ INTERVENTIONS: acute pain assess PQRST monitor effectiveness of analgesia monitor I&O when dehydrated = O2 apply heat to joints as ordered provide rest periods administer fluids oral fluid intake drugs morphine acetaminophen NSAIDS other pharmacological management hydroxyurea sodium cromoglycate folic acid supplement Fe supplement blood transfusion penicillin prophylaxis (prevent infection) pneumococcal vaccination (prevent infection)
readiness for enhanced self-care

client education explain nature of disease adherence to treatment follow up routine CBC count genetic counseling


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Transplantation processed donor cells are injected into the recipient where they find their way to the bone marrow and attach most critical days: first 100 days (post transplantation before transplantation: patient understanding procedure treatment protocol to be used venous access during transplantation: monitor VS maintain sterility watch out for complications after implementation: monitor VS administer meds provide good oral care meticulous skin care observe nursing care Antithymocyte Globulin (ATG): immunosuppressive agent selectively destroys Tlymphocytes gamma globulin from rabbits/horses that have been immunized against human thymocytes indication: aplastic anemia, transplant rejection WHY T CELLS? they attack the bone marrow

gene aberration hemoblastic cell line

excess production of RBC, WBC and platelets

total blood volume, blood viscocity

erythrocytosis excessive of RBCs Hct higher than 55% can be primary, secondary, or relative











CLINICAL FEATURES: blood flow headache dizziness sensory deficits (vision, hearing) chest pain viscocity HTN thromboses (major cause of M/M) SOB, especially when lying flat splenomegaly venous stasis pphletora: ruddy appearance to face, especially nose clubbing of fingers dusky appearance to lips and mucous membranes POSSIBLE NURSING DIAGNOSIS: pain r/t effects of altered blood flow in the distal extremities risk for ineffective tissue perfusion r/t sluggish blood flow and risk for thrombosis NURSING CARE: health teaching dangers of smoking ( blood viscocity) adequate hydration prevent blood stasis report signs of thrombosis and bleeding monitor hct and blood counts MANAGEMENT: periodic phlebotomy removal of certain amount of blood goal: blood volume make client non-deficient (no RBC production) special considerations: before explain procedure watch out for tachycardia, clamminess, vertigo VS keep patient supine after watch out for s/sx of iron deficiency, bleeding advice ambulation oral fluid intake VS monitor blood counts ambulate slowly

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conditions in which there are fewer leukocytes than normal results from Neutropenia or Lymphopenia NEUTROPENIA ACUTE POTENTIAL BLOOD DYSCRASIA FAILURE TO PRODUCE ADEQUATE NUMBERS OF NEUTROPHILS 3 (LESS THAN 2000/MM ) FEMALE > MALE SUSCEPTIBLE TO BACTERIAL INVASION AND INFECTION CAUSES: production of neutrophils aplastic anemia d/t meds/toxins metastatic cancer lymphoma leukemia myelodys plastic syndromes chemo radiation therapy destruction of neutrophils hypersplenism (splenomegaly) medication-induced immunologic disease (SLE) viral disease (ex. infection, hepatitis, mononucleosis) bacterial infections CLINICAL MANIFESTATIONS: rapid onset severe fatigue and weakness sore throat buccal and pharyngeal ulcerations abdominal discomfort fever weak and rapid pulse severe chills MANAGEMENT: removal of causative factor disease is usually reversed 2-3 weeks after BMT may be required if condition is not reversed surveillance cultures of blood, throat, sputum, urine, and stool to monitor infections treatment includes colony-stimulating factors (CSF) to neutrophil production given after offending agent is eliminated given before serious infection, prognosis is better broad spectrum antibiotics control of oral and gingival pain meticulous oral care saline rinses local anesthetic gels and gargles soft or liquid food until mouth and gum sores are diminished NURSING DIAGNOSIS: risk for infection ineffective protection initiate neutropenic precautions by preventing complications MANAGEMENT: environment strict medical asepsis maintain cleanliness

fresh flowers are not allowed inside the room maintain cleanliness of room isolation precaution patient wear mask (avoid inhaling infections) hygiene (meticulous care in terms of hygiene) check VS most especially temperature (for presence of fever) diet of patient not allowed to eat fresh unpeeled fruits, microbial diet hydration status IV status presence of phlebitis watch out for signs of inflammation



FOR NORMAL CLOTTING AND LYSIS TO OCCUR: intact blood vessels adequate platelets sufficient amount of the 12 clotting factors well-controlled fibrinolytic system DIAGNOSTICS: complete health history physical exam lab tests most crucial data to pinpoint the cause of hemorrhage SIGNS AND SYMPTOMS: petechiae tiny hemorrhagic spots caused by intradermal (ID) or submucosal bleeding ecchymosis large, blotchy, SQ hemorrhagic areas hematoma subdermal hemorrhage hemarthrosis bleeding in the joints

most common cause of hemorrhagic disorders decrease in circulating platelets (less than 100,000/uL) 4 major reasons why it occurs: in platelet production platelet lifespan blood pooling of spleen dilution of the bloodstream 50,000 uL = minor trauma leads to bleeding 10,000 uL = spontaneous bleeding even without trauma TYPES: Immune/Idiopathic Thrombocytopenic Purpura Thrombotic Thrombocytopenic Purpura Secondary Thrombocytopenic Purpura Disordered Platelet Distribution

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THROMBOTIC THROMBOCYTOPENIC PURPURA CAUSES: toxins released after bacterial invasion causes widespread thrombosis platelet tend to clump together and infiltrate to tissues leading to ischemia SECONDARY THROMBOCYTOPENIA CAUSES: usually secondary to drugs/meds (thiazides, aspirin, NSAIDs, Sulfonamides, Tagamet, Lanoxin, Lavix, Heparin, Morphine, Tegretel, Vit C and E) spices (ginger, garlic) infections (bacterial or viral) bone marrow disorders chemotherapy, radiation therapy IMMUNE THROMBOCYTOPENIC PURPURA IDIOPATHIC THROMBOCYTOPENIC PURPURA HEMORRHAGIC AUTOIMMUNE DISEASE THAT


ETIOLOGY AND RISK FACTORS: binding of auto-antibodies to antigens on platelet membrane common among women: 20-40 years old children: after a viral infection onset PATHOPHYSIOLOGY:
platelets perform normally in the blood platelets are covered with antibodies (recognized as foreign) destroyed by macrophages when reaching the spleen and liver total in number of platelets platelets only survive from 1-3 days or less

MEDICAL MANAGEMENT: goal: safe platelet count high-dose corticosteroids SE: moon-faced, buffalo-hump, susceptible to stress suppresses platelet destruction plasmapheresis (short-term therapy) IV gamma globulin ( platelet count) splenectomy 60-80% results in complete/permanent remission immunosuppressive drugs Vincristine Vinblastine Azathioprine NURSING MANAGEMENT: health teaching signs of disease exacerbation side effects of drug being taken by client (corticosteroids) prevention of bleeding soft bristle toothbrush electric shavers care with trimming nails avoid rectal temperature taking, enemas and suppositories avoid IM injection avoid activities that ICP avoid sexual intercourse if platelet count is 3 <10,000mm if for vernipuncture, draw all blood for lab work with one vernipuncture

X-linked genetic bleeding disorder deficiency of coagulation factors found usually among males detected during early childhood, usually in toddler age (bleeding at 1 year old)

DEADLY DILLEMAS: spontaneous hemorrhage, cerebral, GIT, GUT, diaphragm nerve pain, extremity anesthesia and paralysis SIGNS AND SYMPTOMS: (GSHEEEP) Gingival bleeding Splenomegaly (tend to eat foreign platelets) Heavy menses (menorrhagia) Ecchymosis Epistaxis Easy bruising Petechiae DIAGNOSIS: platelet count below 100,000 prolonged bleeding time with normal coagulation time capillary fragility (+) platelet antibody screening bone marrow aspirate containing normal or number of megakaryocytes (where platelets came from)

TYPES: Hemophilia A classic factor VIII Hemophilia B Christmas disease factor IX (christmas factor) von Willebrands disease vWF carries factor VIII into the circulation and promotes platelets function CLASSIFICATION: Severe =<1% of normal factor level (spontaneous hemorrhage) Moderate =<1% - 5% Mild MANIFESTATIONS: Bleeding (HIS SHEP) Hemarthrosis Internal bleeding Surgical procedure that result in excessive bleeding

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Spontaneous hematuria Hematoma Epistaxis, bleeding of other mucous membranes Pain MEDICAL MANAGEMENT: fresh frozen plasma (contain clotting factors) factor VIII and IX concentrates during acute bleeding, preventive measure when undergoing procedures Aminocaproic acid fibrinolytic enzyme, slows the dissolution of clots Desmopressin Vasopressin analog induces transient rise in factor VIII NURSING MANAGEMENT: applying pressure on minor trauma splinting with joint or muscle hemorrhages avoid injections and all other invasive procedures perform only after administration of appropriate factor replacement analgesics for pain warm bath may improve and lessen pain during hemorrhagic episodes, rest on accentuate bleeding cold compress is used wear necessary medical band for identification during hemorrhagic episodes careful assessment for emergent complications frequent VS monitoring watch out for: respiratory distress and altered LOC assist patient in coping and accepting condition health teaching activity restriction self-care measures safety at home and workplace avoid any agents that interfere with platelet aggregation (aspirin, NSAIDs, etc) dental hygiene

CONDITIONS THAT MAY PRECIPITATE DIC: shock cirrhosis snake bite glumerulonephritis septicemia retention of a dead fetus trauma heat stroke PATHOPHYSIOLOGY:


complex syndrome of activated coagulation loss of balance between the clotting and lysing system of the body consumptive coagulopathy DIC starts with excessive clottings clotting factors depleted excessive bleeding ensues CATEGORIES OF RISK FACTORS: (STIDS) Secondary response to primary insult Tissue coagulation factors Infection (most common cause) gram (-) septicemia typhoid fever rocky mountain spotted fever Damage to vascular endothelium Stagnant blood flow

MANIFESTATIONS: classified: acute, subacute, and chronic characterized by widespread bleedings thrombosis bleeding at 3 unrelated sites DIAGNOSIS: screening assays confirmatory assays ( partial thromboplastic time) presence of DIC pathology MANAGEMENT: goals: identification and correction of precipitating cause or problem reestablishing hemostasis by replacing missing blood components (depends on type) supportive therapy to control manifestation of hemorrhage and thrombosis control bleeding gabexate and aprotinin IV heparin administration used to control thrombosis may also cause bleeding to dissolve the clot NURSING MANAGEMENT: assess all body systems integumentary respiratory: tachypnea, hemoptysis, orthopnea, pulmonary infarction cardio: tachycardia and hypotension GI: abdominal distention, hematemesis (+), guaiac test (test for blood in stool) GUT: hematuria, oliguria neuro: vision changes, dizziness, HA, changes in mental status general goals: monitor and quantify blood loss provide supportive therapy with blood components resolve manifestations of hemorrhage control further bleeding

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CONVENTIONAL UNITS 0.5%1.5% of red cells M: 42%52% F: 35%47% M: 13.517.5 g/dL F: 11.515.5 g/dL

INTERPRETATION = in bone marrow activity = acute leukemia, late stage of severe anemias = associated with shock = severe anemia, pregnancy anemia, acute massive blood loss = polycythemia, COPD, failure of oxygenation d/t CHF, people in living in high altitudes = various anemias, preggy, severe/prolonged hemorrhage, excessive fluid intake



Leukocyte Neutrophils

T: 4,50011,000/ cu mm 45-73% (2,500-7,500 mm3)

Eosinophils Basophils

0%4% 0%1%





Platelet count

150,000 450,000/cu mm3

= acute infections, trauma, surgery, leukemia, malignant disease, necrosis = viral infections, bone marrow suppressions, primary bone marrow disease = allergy, subacute infections = stress, use of meds (ACTH) = acute leukemia and following surgery or trauma = allergic reactions, stress, allergy, use of corticosteroids = viral and some bacterial infections, hepatitis = aplastic anemia, SLE, immunodeficiency including AIDS = viral infections, hemolytic disorders = use of corticosteroids, RA, HIV infection = malignancy = thrombocytopenic purpura, aplastic anemia

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