Blood Cell & Clotting Abnormalities PDF

BLOOD CELL & CLOTTING ABNORMALITIES Dorean Behney Hurley MSN, RN, CCRN  COMPONENTS OF BLOOD Leukocytes (WBCs) Thrombocytes (Platelets) Erythrocytes (RBCs) Plasma  All cells derive from a single pool of pluripotent (multipotent) stem cells of bone marrow  Lymphoid stem cells...

BLOOD CELL & CLOTTING ABNORMALITIES Dorean Behney Hurley MSN, RN, CCRN  COMPONENTS OF BLOOD Leukocytes (WBCs) Thrombocytes (Platelets) Erythrocytes (RBCs) Plasma  All cells derive from a single pool of pluripotent (multipotent) stem cells of bone marrow  Lymphoid stem cells  Myeloid stem cells BONE MARROW GIVES RISE TO ALL BLOOD CELLS INTRODUCTION A&P REVIEW - ERYTHROCYTES MATURE RBCS  Biconcave disk  Hemoglobin  Carries oxygen to body tissues  Color  Vitamin and minerals needed for HGB synthesis  Iron - 67% of iron is in the form of HGB  Iron is recycled  Folate– needed for DNA & RNA synthesis for RBC production  Vitamin B12 – needed for DNA synthesis, nuclear maturation, normal cell division & prevents myelin breakdown  A&P REVIEW: ERYTHROPOIESIS  Proerythroblasts  Produced by pluripotent stem cells in bone marrow  Precursor cells of RBCs  1 wk stem cell to reticulocyte  Enters blood as reticulocyte  Matures into erythrocyte in 24-48 hrs.  RBC production = destruction LABORATORY TESTS  RBC – total #  HGB  14-16.5 g/dL - men  12-15 g/dL - women  HCT  40-50% - men  37-47% - women  Reticulocytes – index of the rate of RBC production  1-1.5%  MCV  Microcytic  Macrocytic  MCH  MCHC  Hypochromic Do not  Normochromic memorize # Labwork helpful in diagnosis of anemia MANIFESTATIONS OF ANEMIA  ANEMIA OF ACUTE BLOOD LOSS  Volume loss causes a decrease in venous return  Low BP, decreased CO & decreased central pressure  Diverts blood to vital organs kidney RAS conserve salt & H2O  sympathetic nerve activation blood vessels constrict  5 days for progeny of hematopoietic stem cells to differentiate fully  RBC count returns to normal 3-4 weeks (need adequate iron stores)  PERNICIOUS ANEMIA VITAMIN B 12 (COBALAMIN)-DEFICIENCY  Megaloblastic anemia  Causes:  Gastric/Stomach disorders  Terminal ileum disorders  Strict vegetarian diet  Symptoms – usual anemia symptoms  Changes on mucosal cells  Neurologic deficits  Diagnosis and treatment  CBC – elevated MCV, normal MCHC & reticulocyte count  Low B 12 levels  Parietal cell & intrinsic factor antibodies  FOLIC ACID ANEMIA  Megaloblastic anemia  Causes  Dietary deficiency  Malabsorption  Drug interactions  Pregnancy  Symptoms  Diagnosis  MCV & normal MCHC  Serum folate levels IRON DEFICIENCY ANEMIA  Microcytic-hypochromic anemia  Causes  Dietary deficiency  Increased iron demands  Loss of iron from bleeding  Symptoms  Lab  lowiron & ferritin levels  Low HGB & HCT  MCV & MCHC ANEMIA OF CHRONIC DISEASE SEEN IN CHRONIC SYSTEMIC DISEASES  Results from decreased life span & erythropoiesis T cell activation & production of cytokines  Altered iron metabolism  Renal failure patients  Labs  Normocytic (MCV) & normochromic (MCHC)  Low reticulocyte count  Low transferrin (destruction from cytokines)  Mild symptoms APLASTIC ANEMIA DISORDER OF PLURIPOTENTIAL BONE MARROW STEM CELLS  Pancytopenia  Causes  High does of XRT  Chemicals & toxins that suppress hematopoiesis  Infections  Idiopathic 2/3rd of cases  Signs  Treatment  Initially require transfusions  Bone marrow or stem cell tx  Immunosuppressive therapy  HEMOLYTIC ANEMIA  Premature destruction of RBC  Causes  Hereditary  Aquired – mechanical trauma, transfusion reaction, infections  S&S  Easyfatigability, dyspnea, increase rate & depth  Jaundice  Lab  Normocytic (MCV) & normochromic (MCHC)  Increased reticulocyte count  Coombs test (for dx of immunohemolytic) SICKLE CELL ANEMIA INHERITED DISORDER OF AN ABNORMAL HGB(HEMOGLOBIN S)  Inherited recessive trait  Sickle cell trait (one HbS gene)  Sickle cell disease (two HbS genes)  HbS polymerizes when deoxygenated semi solid gel  Results in:  Chronic hemolytic anemia  Blood vessel occlusion  Tissue ischemia  Pain SICKLE CELL ANEMIA  Factors associated with sickling & occlusion  Complications  Vaso-occlusive pain  Acute chest syndrome  Prone to infections POLYCYTHEMIA VERA Primary  Cause  Labwork  Elevated– RBC, H&H, WBC & Platelets  Decreased – iron levels  Complications & symptoms  Splenomegaly  Hypertension  Viscosity – venous stasis  Dusky redness to lips, fingernails & mucous membranes  Thromboembolism, hemorrhage from rapidly produced defective platelets Secondary  Causes AGE RELATED ISSUES REGARDING RBCS Elderly  Anemia common in elderly  Increases morbidity & mortality if untreated  Usually asymptomatic in iron deficiency & chronic disease  Stress bleeding RBCs not replaced as quickly # progenitor cells decrease with age  Decrease in growth factors stimulating new cells  Erythropoietin issues LEUKOCYTE ORIGINATION  Granulocytes Precursor  Neutrophils cells  Basophils  Eosinophils  Agranulocytes  Lymphocytes  B lymphocytes  T lymphocytes  Natural killer cells  Monocytes WBC count 4.5 – 10.5 cell/uL  Leukopenia  Leukocytosis  NEUTROPENIA  Low neutrophil count - levels less than 1000/ul  Increases risk of infection  Causes  Clinical manifestations  Ulcersof mouth and skin lesions  Recurrent infections  Fever  Treatment  Prevent infection – neutropenic precautions  Treat infections until bone marrow recovers  Administration of recombinant human G-CSF LEUKEMIAS AND LYMPHOMAS Lymphoid precursor Pre-T Pre-B lymphoblast lymphoblast Naïve B cell Plasma cell  LEUKEMI Leukemia AS  Lymphocytic – ALL, CLL  Involve immature lymphocytes & their lymphoid progenitors  Myelogenous – AML, CML  Involve myeloid stem cells of bone marrow  ACUTE LEUKEMIAS Blast cells – AFFECT IMMATURE BLOOD FORMING CELLS  ALL - B or T lymphoblasts  Chromosomal changes  Affect normal blood cell development  Affects mostly children  AML – myeloid precursor cells of BM  Acquired genetic alteration  Inhibits terminal myeloid differentiation  Increased blast cells formation  Affects mostly older adults AML & ALL - SYMPTOMS PRESENT SIMILARLY ABRUPT ONSET OF FATIGUE, LOW GRADE FEVER, NIGHT SWEATS, & WEIGHT LOSS  Bone marrow suppression  Rapid proliferation & hypermetabolism of leukemic cells  Leukemic infiltration  Bone pain  Lymphadenopathy, splenomegaly, hepatomegaly (more common in ALL)  CNS symptoms (more common in ALL)  Infection  Leukostasis – blast count > 100,000 Diagnosis & Treatment CHRONIC LYMPHOCYTIC LEUKEMIA CA INVOLVING PROLIFERATION OF MORE FULLY DIFFERENTIATED LYMPHOID CELLS  CLL - Clonal malignancy of B lymphocytes  Failure of B cells to mature into plasma cells  Cause unknown  Usually asymptomatic  Diagnostics  Elevated WBC count  75-98% are lymphocytes  Specific testing for CD 5+, CD 19+, CD 20+, CD 23+  CHRONIC MYELOGENOUS LEUKEMIA PROLIFERATION OF MORE FULLY DIFFERENTIATED MYELOID CELLS  CML- affects pluripotent hematopoietic progenitor cell  Excessive proliferation of marrow granulocytes, erythroid precursors & megakaryocytes  Philadelphia chromosome  Clinical course – 3 phases  Chronic phase  Short accelerated phase  Terminal blast phase  # myeloid precursors  in accelerated symptoms  Poor prognosis – 3-6 months  Diagnostics  Elevated WBC  BCR-ABL gene product LYMPHOMAS  Malignancies of the peripheral lymphoid tissues  Solid tumors containing neoplastic lymphoid cells HODGKIN LYMPHOMA CANCER OF LYMPHOID TISSUE – SOLID TUMORS  Cause unknown - ? Mutation of B cell germinal or post- germinal center of lymph node  Reed-Sternberg cells found  Large, atypical mononuclear tumor cells  Interspersed among normal lymph tissue and lymph organs  Starts in a single node or chain of nodes HODGKIN LYMPHOMA  Symptoms  Painless enlargement of a lymph node or group of nodes  1/3 will report systemic symptoms  Advanced disease leads to immunologic defect in cell mediated response at risk for infections  Diagnosis – Lymph node biopsy  Staging determined by # of nodes involved  NON – HODGKIN LYMPHOMAS -LACK RS CELLS, AFFECT MULTIPLE NODES & SPREADS UNPREDICTABLY  B cell neoplasms – 85% of NHL  Burkittlymphoma – associated with EBV  Mantle cell lymphoma – Immunoglobulin gene arrangements  MALT lymphomas of stomach associated with chronic H pylori  T cell neoplasms  Human T-cell lymphotropic virus of southwest Japan MULTIPLE MYELOMA  Pathogenesis: B lymphocytes turn into malignant plasma cells  Osteolytic bone lesions  Cause unknown  Manifestations  Diagnosis  Bone marrow aspirate  Lytic bone lesions  Lab work  M protein spike serum  Bence Jones proteins in urine PLATELETS AKA THROMBOCYTES  Platelet count 150,000-400,000 uL  Remain in bloodstream, but can be stored in spleen  Thrombopoietin  Structural components  Granules become mediators when injury to vessel occurs  Alpha granules  Dense granules  Glycoprotein IIb/IIIa  Phospholipids  Platelets responsible for first step of hemostasis  THROMBOCYTOPENIA  Platelet count < 100,000,  Risk for bleeding  Spontaneous bleeding occurs when < 10,000-15,000/uL  Causes – anything that decreases platelets  Bone marrow issues  Infections  Excessive platelet destruction – from antiplatelet antibodies  Massive blood transfusion protocol – dilutes platelets  Drug induced  Treatment THROMBOCYTOSIS  Asymptomatic until platelet count over 1,000,000 u/L  Increased risk for clot formation from platelet adhesion Conditions Associated with Increased Platelet Function Increased Platelet numbers Endothelial injury Reactive disorders Atherosclerosis -splenectomy Elevated lipids & cholesterol -cancer Smoking -chronic inflammatory disorders Myeloproliferative disorders -polycythemia vera BLOOD CLOTTING MECHANISM HEMOSTASIS  Vessel spasm < 1 minute  Thromboxane (TXA2) & neural reflexes  Formation of platelet plug (primary)  Platelet adhesion  vWf binds to platelet receptors & collagen fibers  Activated platelets release granules  Calcium  ADP & TXA2  Create primary hemostatic platelet plug HEMOSTASIS  Blood coagulation-(secondary hemostatic plug)  Pathways activation Factor X  Convert prothrombin thrombin  Conversion fibrinogen fibrin  Clot Retraction  Actin & myosin in platelets  Clot Dissolution/fibrinolysis  Beginssoon after clot is formed  Plasminogen  Trapped in clot  Activated by tPa - tissue plasminogen activator  Converts to plasmin digests fibrin  IMPAIRED HEMOSTASIS Click to edit Master text Click to edit Master text Vitamin K deficiency Liver Disease styles styles  Fat soluble vitamin  Leads to diminished  Prothrombin production of clotting  Factors VII, IX, X factors  Protein C & S  Factor VII, IX  Plasminogen & α -  Causes 2  Bulimia antiplasmin  Parental nutrition w/  Clotting regulators antibiotics  Antithrombin, fibrinogen  Protein C, Protein S  Treatment  Vitamin K injections  Diminished thrombopoietin  Fresh Frozen Plasma (FFP) TRIAD OF VIRCHOW RISK FOR DEVELOPING SPONTANEOUS THROMBI  Injury to vessel endothelium  Plaques, turbulent flow, toxins  Abnormalities of blood flow  Turbulence  Stasis  Hypercoagulability of blood  Primary  Secondary