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Hematologic disorders
 

Hematologic disorders

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    Hematologic disorders Hematologic disorders Presentation Transcript

    •  The blood and the blood forming sites, including the bone marrow and the reticuloendothelial system Blood › Plasma › Blood cells Hematopoiesis
    • Blood Adult: •Female 4-5L •Males 5-6L
    •  Transport of: › Gases, nutrients, waste products › Processed molecules › Regulatory molecules Regulation of pH and osmosis Maintenance of body temperature Protection against foreign substances Clot formation
    • Antibodies & complements–part of immune systemClotting factorOnce activated, convertedto fibrin (threadlike proteinthat forms blood clot
    •  Liquid part of blood › Pale yellow made up of 91% water, 9% other Colloid: Liquid containing suspended substances that don’t settle out › Albumin: Important in regulation of water movement between tissues and blood › Globulins: Immune system or transport molecules › Fibrinogen: Responsible for formation of blood clots
    •  Erythrocyte: RBC Leukocyte: WBC › Neutrophil › Monocyte › Eosinophil › Basophil › Lymphocyte: T lymphocyte and B lymphocyte Thrombocyte: platelet
    •  Hematopoiesis or hemopoiesis: › Process of blood cell production › Fetus: liver, thymus, spleen, lymph nodes, red bone marrow › After birth: red bone marrow, for WBC in some lymphatic tissues Stem cells (hemocytoblast): All formed elements derived from single population › Proerythroblasts: Develop into red blood cells › Megakaryoblasts: Develop into platelets
    • 19-10
    •  Structure › Biconcave, anucleate Components › Hemoglobin (makes it red in color) › Lipids, ATP, carbonic anhydrase Function › Transport oxygen from lungs to tissues and carbon dioxide from tissues to lungs
    • Unable to divide, grow, or synthesize proteins Wear out in 100 to 120 days Removed by phagocytes in the spleen or liver New RBCs made by stem cells in bone marrow Production increases when oxygen level decrease or during pregnancyCopyright © 2003 Pearson Education, Inc. publishing as Benjamin Cummings
    •  Consists of: › 4 globin molecules: Transport carbon dioxide (carbonic anhydrase involved), nitric oxide  2 alpha and 2 beta globin chains › 4 heme molecules: Transport oxygen  Each heme contains 1 iron  Iron is required for oxygen transport › Hemoglobin + oxygen = bright red › Hemoglobin with no oxygen = darker red
    • Erythrocytes: Levels in Blood Live only four (4) months or ~120 days Average RBC count: Males: 5.4 million/mm3 Females: 4.8 million/mm3 Outnumber white blood cells 1000:1Copyright © 2003 Pearson Education, Inc. publishing as Benjamin Cummings
    •  Happens in red bone marrow Components required: › Precursor cells: PROERYTHROBLAST › Proper microenvironment › Adequate supplies of iron, vitamin B12, folic acid, protein, pyridoxine and traces of copper Erythropoietin: › Hormone to stimulate RBC production
    •  During cell division, the process requires B vitamins folate and B12, necessary for synthesis of DNA. Iron is required for production of hemoglobin RBC production is triggered by low blood oxygen levels
    •  Cytotoxic cells Helper cells Memory cells Suppressor cells
    •  IgG › chronic infxn, transplacental; IgA › external secretions; “mucosal paint” › through breastfeeding IgM › acute infxn; 1O response Ab, 1st produced by infants IgE › parasitic / allergic reactions IgD › no specific function; antigen-binding
    •  factor I (fibrinogen) factor II (prothrombin) – Vit K dependent factor III (tissue thromboplastin) factor IV (calcium) factor V (proaccelerin) factor VI (no longer considered active in hemostasis) factor VII (proconvertin) - Vit K dependent factor VIII (antihemophilic factor) factor IX (plasma thromboplastin component; Christmas factor) - Vit K dependent factor X (stuart factor) - Vit K dependent factor XI (plasma thromboplastin antecedent) factor XII (hageman factor) factor XIII (fibrin stabilizing factor).
    • Spleen › Left upper quadrant of the abdomen behind abdomen › Produces RBC during fetal development › Filter function – remove old defective cells from circulation and breaks down RBC and recycles the iron to bone marrowLiver › Receives blood from the spleen through portal circulation › Produce procoagulants necessary for hemostasis and blood coagulation › Storage of iron and B12 › Detoxify drugs › Kupffer cells carry out phagocytosis › Bile production from erythrocyte destruction
    • Lymphatics Lymph capillaries › Carries lymph to the subclavian vein in the chest Lymph nodes › Small rounded structures that filter bacteria and foreign particles
    •  Reduction below normal in the # of RBC, the quantity of hemoglobin and volume of RBC (hematocrit) Result from: › ↓RBC production › ↑RBC destruction › Acute or chronic blood loss
    •  Three broad categories1. Loss of RBC- occurs with bleeding2. Decreased RBC production › IDA › FADA › Thalassemia3. Increased RBC destruction
    •  Normocytic › Normal RBC size › IDA Macrocytic › Large RBC due to impaired division of RBC precursor cells › Cobalamin and folate deficiency Microcytic › Small RBC Normochromic › Normal hemoglobin concentration Hypochromic › Decrease hemoglobin concentration Hyperchromic › Increased hemoglobin concentration
    •  Iron Deficiency Anemia › dietary intake of iron is inadequate to produce hemoglobin › Most common type of anemia › May occur with removal of the duodenum › Associated with chronic blood loss.
    •  Iron Deficiency AnemiaEtiologic Factors 1. Bleeding- the most common cause 2. Mal-absorption 3. Malnutrition 4. Alcoholism
    •  Iron Deficiency AnemiaPathophysiology › The body storage of iron decrease, leading to depletion of hemoglobin synthesis › The oxygen carrying capacity of hemoglobin is reduced tissue hypoxia
    •  Iron Deficiency AnemiaAssessment Findings1. Pallor of the skin and mucous membrane2. Weakness and fatigue3. General malaise4. Pica
    • Assessment Findings5. Brittle nails6. Smooth and sore tongue7. Angular cheilosis
    •  Iron Deficiency AnemiaLaboratory findings1. CBC › Low levels of Hct, Hgb and RBC count2. Low serum iron, low ferritin3. Bone marrow aspiration- MOST definitive
    •  Iron Deficiency AnemiaMedical management 1. Iron replacement 2. Blood transfusion
    • Nursing Management1. Provide iron rich-foods › Organ meats (liver) › Beans › Leafy green vegetables › Raisins and molasses2. Administer iron › Oral preparations tablets- Fe fumarate, sulfate and gluconate › Advise to take iron ONE hour before meals › Take it with vitamin C › Monitor for adverse reaction like pyrosis (heartburn), constipation, diarrhea, dark stool › Continue taking it for several months even with normal iron level
    • Nursing Management2. Administer iron › Oral preparations- liquid  It stains teeth  Drink it with a straw › Stool may turn blackish- dark in color › Advise to eat high-fiber diet and to ↑fluid intake to counteract constipation
    • Nursing Management2. Administer iron › IM preparation  Administer DEEP IM  Include 0.5ml air in syringe to clear iron from the needle  Use the Z-track method › Avoid vigorous rubbing › Can cause local pain and staining Note: › Iron should be taken for 2-3 mos after Hb level returns to normal
    • 3. Administer packed red blood cell transfusion if patient is symptomatic
    •  Normally hemoglobin contain 4 globin chains Decreased production of hemoglobin due to abnormal hemoglobin synthesis Reduced production or no production of 1 of the globin chains that make up hemoglobin Microcytic and hypochromic Autosomal recessive genetic disorder common among Mediterranean people. Chronic bone marrow hyperplasia Altered globin synthesis of hemoglobin Treatment causes chronic iron toxicity
    •  Thalassemia minor › Has 1 thalassemic and 1 normal gene with mild clinical manifestations › Requires no tx Thalassemia major › Has 2 thalassemic genes causing severe conditions
    • Assessment:1. Skin: pale/jaundiced2. Splenomegaly and hepatomegaly3. Thickened cranium and maxillary sinus space from bone marrow hyperplasia
    • Diagnostics:1. CBC 1. Anemia lower Mean corpuscular volume2. Hemoglobin electrophoresis
    • Nursing Management1. Administer BT and chelation therapy (remove heavy metals) to reduce iron overload2. Monitor for transfusion reactions or diseases acquired through transfusions3. Instruct about tx, medications and physical energy conservation techniques4. Strengthen client support and family systems.
    •  Life-threatening stem cell disorder with many possible etiological mechanisms Decreased number of RBC as well as WBC and platelets Characterized as: › hypoplastic( incomplete development of a tissue or organ) › Fatty bone marrow › Pancytopenia (reduction in # of RBC, WBC and platelets)
    • CAUSATIVE FACTORS1. Environmental toxins- pesticides, benzene2. Certain drugs › Chemotherapeutic agents › Chloramphenicol › Phenothiazines › Sulfonamides3. Heavy metals4. Radiation
    • Pathophysiology Toxins cause a direct bone marrow depression ↓ acellular bone marrow ↓ decreased production of blood elements
    •  ASSESSMENT FINDINGS1. fatigue2. pallor3. dyspnea4. bruising5. splenomegaly6. retinal hemorrhages
    •  LABORATORY FINDINGS1. CBC › decreased blood cell numbers2. Bone marrow aspiration confirms the anemia- hypoplastic or acellular marrow replaced by fats
    •  Medical Management1. Bone marrow transplantation2. Immunosupressant drugs3. Rarely, steroids4. Blood transfusion
    •  Nursing management1. Assess for signs of bleeding and infection2. Pancytopenia plan of care to prevent complications like infection and bleeding. 1. Private room 2. Strict hand washing 3. Minimizing invasive procedures3. Provide client and family with support for lengthy hospitalization and tx
    •  abnormally large RBC secondary to impaired DNA synthesis due to deficiency of Folic acid and/or vitamin B12
    • 1. Folic Acid deficiency › Folate is required for DNA synthesis in erythrocyte formation › Without neurologic involvementCausative factors1. Alcoholism2. Mal-absorption3. Diet deficient in uncooked vegetables4. Use of oral contraceptives
    • Pathophysiology of Folic acid deficiency Decreased folic acid ↓ impaired DNA synthesis in the bone marrow ↓ impaired RBC development impaired nuclear maturation but Cytoplasmic maturation continues ↓ large size
    • Assessment (Folate Deficiency)1. Poor nutrition2. Alcohol abuse3. Anorexia4. Impaired absorption in the small intestine5. Undergoing hemodialysis (folate is dialyzable)6. Certain drugs can block folate absorption 1. Oral contraceptives 2. Antiseizure drugs (phenytoin) 3. Antibiotics
    • Diagnosis (Folate deficiency)1. CBC 1. ↓ RBC, hgb & hct and ↑ MCV2. Serum folate levels are low3. Serum cobalamin is normal
    • Nursing Management1. Promote compliance with replacement therapy (1-5 mg oral folate/day)2. Administer prenatal vitamins in pregnancy3. Instruct on food high in folate such as leafy greens, liver, citrus fruits, nuts and grains
    • Normal Condition:Parietal cells of stomach secreted intrinsic factor to absorb ingested cobalamin (vit.b12)
    • Vitamin B12 deficiency (Pernicious Anemia) › Lack of intrinsic factor (needed for B12 absorption) › Common among Northern European ancestry over age of 40 and young African American Causative factors1. Strict vegetarian diet2. Gastrointestinal malabsorption3. Crohns disease4. Yrs of Gastritis5. Gastrectomy
    •  Vitamin B12 deficiencyPernicious Anemia Due to the absence of intrinsic factor secreted by the parietal cells Intrinsic factor binds with Vit. B12 to promote absorption
    • Assessment findings1. Weakness2. Fatigue3. Neurologic manifestations are present only in Vit. B12 deficiency 1. Weakness, paresthesia of feet and hands, impaired thought processes4. Coagulation deficiencies
    •  Assessment findingsPernicious Anemia › Beefy, red, swollen tongue › Mild diarrhea › Extreme pallor › Paresthesias in the extremities
    •  Laboratory findings1. Peripheral blood smear- shows giant RBCs, WBCs with giant hypersegmented nuclei2. Very high MCV3. Schilling’s test › Radioactive vit B12 malabsorption is measured by small amount of secreted in urine › When vit B12 us administered with gastric IF parenterally, its absence in urine will diagnose pernicious anemia4. Intrinsic factor antibody test
    •  Medical Management1. Vitamin supplementation Folic acid 1 mg daily2. Diet supplementation Vegetarians should have vitamin intake3. Lifetime monthly injection of IM Vit B12 › w/o cobalamin replacement, patient can die in 1-3yrs.
    •  Since Pernicious anemia results from an inability to absorb cobalamin, dietary intake of the vitamin is not a treatment option, nor is bone marrow transplant
    •  Nursing Management1. Monitor patient2. Provide assistance in ambulation3. Oral care for tongue sore4. Explain the need for lifetime IM injection of vit B12
    •  Decrease in erythrocyte precursor production that occurs in some chronic conditions End-stage renal failure Chronic liver disease Alcohol abuse hypothyroidism
    • Clinical Manifestations ALL ASSESSMENT FINDINGS FOR ANEMIA APPLY
    • Diagnosis:1. CBC2. FERRITIN 1. Iron stores may be high in contrast to IDA
    •  Nursing Management1. Facilitate diagnosis and tx if underlying, contributory condition2. Inform that this type of anemia does not respond to folic acid, iron or vitamin B123. Erythropoietin therapy is administered to a client with anemia related to renal failure
    • 2 SITES OF HEMOLYSIS1. INTRAVASCULAR DESTRUCTION › Occurs within the circulation2. EXTRAVASCULAR DESTRUCTION › Occurs in liver, spleen, or bone marrow
    • CLASSIFICATION OF HEMOLYTIC ANEMIAS1. INTRINSIC HEMOLYTIC ANEMIAS › Usually hereditary › Defects in RBC themselves 1. Abnormal hgb = sickle cell anemia 2. Enzyme deficiencies = G6PD 3. Cell membrane abnormalities
    • CLASSIFICATION OF HEMOLYTIC ANEMIAS2. EXTRINSIC HEMOLYTIC ANEMIAS › Normal RBCs are damaged by external factors › Cause:  antibodies, toxins,  mechanical injury (prosthetic heart valves)  dialysis,  transfusion reaction  trapping of cells within the liver and spleen
    •  A severe chronic incurable hemolytic anemia that results from heritance of the sickle hemoglobin gene. Produces specific mutant form of beta-globin Hypoxia-induced change in RBCs Associated with vascular occlusion and tissue infarction Anemia is caused by accelerated breakdown of abnormal RBC
    •  Causative factor › Genetic inheritance of the sickle gene- Hbs gene
    • Pathophysiology Decreased O2, Cold, Vasoconstriction can precipitate sickling process
    • Pathophysiology Factors ↓ cause defective hemoglobin to acquire a rigid, crystal-like C- shaped configuration ↓ Sickled RBCs will adhere to endothelium ↓ pile up and plug the vessels ↓ ischemia results ↓ pain, swelling and fever
    •  Assessment Findings1. jaundice2. enlarged skull and facial bones3. tachycardia, murmurs and cardiomegaly
    • Assessment Findings Primary sites of thrombotic occlusion: spleen, lungs and CNS Chest pain, dyspnea
    •  Assessment Findings1. Sickle cell crises › Results from tissue hypoxia and necrosis2. Acute chest syndrome › Manifested by a rapidly falling hemoglobin level, tachycardia, fever and chest infiltrates in the CXR
    • During the sickling crisis,the sickling cells clog small capillaries and the resulting hemotasis promotes a self-prepetuating cycle of local hypoxia, deoxygenation of more erythrocytes and more sickling. MEDICAL MGT: Administration of large doses of narcotic analgesics Since pain last 4-6 days
    • Medical Management1. Bone marrow transplant2. Hydroxyurea3. Long term RBC transfusion
    • Nursing Management1. manage the pain › Support and elevate acutely inflamed joint › Relaxation techniques to reduce metabolic needs › analgesics
    • Nursing Management2. Prevent and manage infection › Monitor status of patient › Initiate prompt antibiotic therapy
    • Nursing Management3. Promote coping skills › Provide accurate information › Allow patient to verbalize her concerns about medication, prognosis and future pregnancy
    • Nursing Management4. Monitor and prevent potential complications › Provide always adequate hydration › Avoid cold, temperature that may cause vasoconstriction
    • Nursing Management4. Monitor and prevent potential complications › Leg ulcer Aseptic technique
    • Nursing Management4. Monitor and prevent potential complications Priapism  Sudden painful erection  Instruct patient to empty bladder, then take a warm bath5. Maintain F & E balance to reduce blood viscosity
    •  Glucose-6-phosphate dehydrogenase Results from lack of an RBC enzyme that leads to RBC damage when metabolic needs of RBCs are increased.
    •  A group of X-linked familial hemolytic mutations of the gene Deficiency predisposes to oxidative denaturation of hemoglobin, with resultant red cell injury and lysis. Hemolysis occurs as the result of oxidative stress generated by either an infection or exposure to certain drugs. More common among males of Mediterranean or African descent
    • •Jaundice •HeadacheAssessment •Dizziness •Easy fatigabilty
    • Diagnostic:Use of G6PD assay or screening testQuantification of G6PD levels during nonhemolytic phase
    • Nursing Management1. Provide periods of rest2. Provide adequate hydration3. Administer antibiotics and other tx as ordered to minimize complications.
    •  Refers to an INCREASE volume of RBCs The hematocrit is ELEVATED to more than 55%
    •  POLYCYTHEMIA VERA › Primary Polycythemia  A proliferative disorder in which the myeloid stem cells become uncontrolled  Neoplastic disorder where there is an increased in 3 types of blood cells
    •  POLYCYTHEMIA VERACausative factor › unknown
    •  POLYCYTHEMIA VERA Pathophysiology › The stem cells grow uncontrollably › The bone marrow becomes HYPERcellular and all the blood cells are increased in number
    •  POLYCYTHEMIA VERAPathophysiology › The spleen resumes its function of hematopoiesis and enlarges › Blood becomes thick and viscous causing sluggish circulation › Overtime, the bone marrow becomes fibrotic
    •  POLYCYTHEMIA VERAAssessment findings 1. Skin is ruddy, pruritic due to histamine release 2. Splenomegaly in primary PV only 3. headache, tinnitus 4. dizziness, blurred vision 5. Angina, intermittent claudication, dyspnea and thrombophlebitis
    •  POLYCYTHEMIA VERA Laboratory findings 1. CBC- shows elevated RBC mass 2. Normal oxygen saturation 3. Elevated WBC and Platelets
    •  POLYCYTHEMIA VERAComplications 1. Increased risk for: 1. Thrombophlebitis  Due to hypervolemia & hyperrviscosity,  Tx: active/passive leg exercise and ambulation 2. CVA 3. MI 2. Bleeding due to dysfunctional blood cells
    • POLYCYTHEMIA VERAMedical Management 1. To reduce the high blood cell mass- PHLEBOTOMY 2. Allopurinol 3. Dipyridamole 4. Chemotherapy to suppress bone marrow
    •  Nursing Management 1. Primary role of the nurse is EDUCATOR 2. Regularly asses for the development of complications 3. Assist in weekly phlebotomy 4. Advise to avoid alcohol and aspirin 5. Advise tepid sponge bath or cool water to manage pruritus
    •  Malignant disorders of blood forming cells characterized by UNCONTROLLED proliferation of WBC in the bone marrow- replacing marrow elements . The WBC can also proliferate in the liver, spleen and lymph nodes.
    •  named after the specific lines of blood cells afffected primarily › Myeloid › Lymphoid › Monocytic
    •  The leukemias are named also according to the maturation of cells ACUTE › The cells are primarily immature CHRONIC › The cells are primarily mature or diferentiated
    •  ACUTE myelocytic leukemia ACUTE lymphocytic leukemia CHRONIC myelocytic leukemia CHRONIC lymphocytic leukemia
    •  ETIOLOGIC FACTORS › UNKNOWM › Probably exposure to radiation › Chemical agents › Infectious agents › Genetic
    • PATHOPHYSIOLOGY of ACUTE Leukemia Uncontrolled proliferation of immature cells ↓ suppresses bone marrow function ↓ severe anemia, thrombocytopenia and granulocytopenia
    • PATHOPHYSIOLOGY of CHRONIC LeukemiaUncontrolled proliferation of DIFFERENTIATED cells ↓ slow suppression of bone marrow function ↓ milder symptoms
    •  ASSESSMENT FINDINGS ACUTE LEUKEMIA › Pallor › Fatigue › Dyspnea › Hemorrhages › Organomegaly › Headache › vomiting
    •  ASSESSMENT FINDINGS CHRONIC LEUKEMIA › Less severe symptoms › organomegaly
    • LABORATORY FINDINGS Peripheral WBC count varies widely Bone marrow aspiration biopsy reveals a large percentage of immature cells- BLASTS Erythrocytes and platelets are decreased
    • Medical Management1. Chemotherapy2. Bone marrow transplantation
    • Nursing Management 1. Manage AND prevent infection › Monitor temperature › Assess for signs of infection › Be alert if the neutrophil count drops below 1,000 cells/mm3
    • Nursing Management2. Maintain skin integrity3. Provide pain relief4. Provide information as to therapy- chemo and bone marrow transplantation
    • Read on the following remaining topics:1. Thrombocytopenia2. Disseminated Intravascular Coagulation3. Hemophilia4. Von Willebrand’s disease5. Neutropenia
    • On September 12, 2011, we will have our UNIT EXAM on the following topics: Fluids and Electrolytes And Hematologic Disorders
    • NEXT TOPIC TO BE DISCUSSED WOULD BE: CARDIOVASCULAR DISORDERS AND PERIPHERAL DISORDERS BY S’ CLAY ENDOCRINE DISORDERS BY M’ LUDY