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Hematology

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  1. Hematology By Joyce Smith RN BSN

  2. Introduction • Red Blood Cell Disorders • White Blood Cell Disorders • Coagulation Disorders • Clotting Factor Disorders

  3. RBC Destruction: Sickle Cell Disease • Hereditary, chronic form of anemia • Abnormal sickle or crescent-shaped erythrocytes are present • D/t abnormal Hemoglobin S (Hb S) in the RBCs • High presence of disease in Mediterranean & African population & African American population in US

  4. Sickle Cell Disease • Sickle Cell Crisis • Exact cause of sickling crisis unknown • 2 factors 1. Hypoxia caused by  O2 tension in blood 2.  blood viscosity/ plasma volume,  hydrogen ion concentration, &  plasma osmolality, caused by concentration of cells & dehydration

  5. Sickle Cell Crisis • Sickle cell interferes with O2 transport • Obstruction of capillary blood flow • Causes fever & severe pain in joints & abdomen • Crisis is precipitated by  O2

  6. Diagnostic Tests • Stained blood smear • Sickle cell observed • Sickle cell preparation of blood • Sickling noted after deoxygenation • Sickle solubility test • Turbid solution indicates presence of Hb S

  7. Diagnostic Tests • Hemoglobin electrophoresis • Presence of Hb S & Hb A indicates sickle cell trait < 40% Hb S • Presence of Hb S only indicates sickle cell anemia 85-95% • Erythrocyte life span • Decreased 10 to 20 days

  8. Hemolytic anemia Pallor Jaundice Fatigue Irritability Extensive Sickling Vaso-occlusive or thrombotic crisis Aplastic Crisis Sequestration Crisis Hyperhemolytic Crisis Clinical Manifestations

  9. Health History • Previous crisis • Precipitating events • Severity • Usual treatment • Review of activities last 24 hours • Food & fluid intake • Exposure to temperature extremes • Type of clothing worn • Meds taken, ingestion of alcohol & recreational drugs • Exercise, trauma, stress

  10. Health History • Activity toleration • Fatigue • Activity intolerance • ADLs • Sleep & rest pattern • Climbing stairs • SOB • Measure on Likert scale 0-10

  11. Assess • Skin • Jaundice or Pallor, ulcerations • Skeletal structures • Joint swelling, disproportionably long arms & legs, skeletal fragility • GI function • Enlarged liver & spleen

  12. Assess • Comfort • Bone, abdominal pain • Cardiac Function • Systolic murmurs, tachycardia, dysrthythmias, cardiac enlargement, hyportension,  pulse pressure • Respiratory Function • Dyspnea, acute respiratory distress

  13. Assess • Sensory Function • Altered LOC, abnormal pupillary response, abnormal reflexes • Renal Function • Decreased UO, edema • Sexuality • Delayed sexual maturity

  14. General Management • Supportive Care • Rest • O2 therapy • IV fluids & e’lytes • Analgesics • Sedatives • Blood transfusion • Genetic counseling

  15. Drug Therapy • Experimental use of antisickling agents • Hydroxyurea • Erythropoientin • Nitric Oxide • Flurocor • Oral administration • Folic acid &/or Fe • Bone Marrow Transplant • Gene Replacement Therapy

  16. Immunohemolytic Anemiaor Autoimmune Hemolytic Anemia •  RBC hemolysis due to attack by own immune system • Attacks antigen on RBC cell membrane • Against universal component of Rh system • Immune system is not able to recognize self as normally would • Cause may be infections, drug reactions, and certain cancers • Hemolytic anemia of newborn can occur with a Rh neg mother and Rh positive baby • Blood transfusions can also cause a problem

  17. Immunohemolytic Anemiaor Autoimmune Hemolytic Anemia • Signs and symptoms • Pallor • Fatigue • Tachycardia • Shortness of breath • Hypotension

  18. Treatment • Glucocorticoids for mild to moderate immune suppression • Cytoxin, Imuran • Plasma exchange therapy • Splenectomy • Transfusion limited or no benefit

  19. PERNICIOUS ANEMIA or Vitamin B 12 Deficiency

  20. Vitamin B12 Deficiency • Rare chronic progressive condition • Abnormal formation & function of RBCs • Inadequate intake or inability to absorb Vitamin B12 • Fatal if not treated

  21. A & P • Can be results of atrophied parietal cells in the stomach • Absence of intrinsic factor (IF), preventing absorption of B12 • IF secreted by parietal cells gastric mucosa • B12 absorbed in distal ileum • Digested B12 attached to IF in stomach, able to pass into blood stream in ileum

  22. A & P • If not attached to IF, B12 passed through the body through the stool • Insidious onset, several months for symptoms to manifest

  23. Symptoms • Affect 3 major body systems • Hematopoietic system • Fatigue • Dizziness • Ringing in ears • Pale or yellowish skin • ^ HR, enlarged heart with murmur • Chest pain

  24. Symptoms • GI system • Sore, beefy red tongue • Loss of appetite, Weight loss • Diarrhea & abd cramping

  25. Symptoms • Nervous system • Numbness, tingling, & burning in arms, legs & feet • Muscle weakness • Difficulty & loss of balance while walking • Changes in reflexes • Irritability, confusion & depression

  26. Diagnostic Tests • CBC abnormally large RBCs, may be abnormal shaped, retic ct. low • WBC may be decreased • Gastric analysis: NG inserted & gastric contents aspirated, acholohydria • Gastorscopy with biopsy: assess parietal cell atrophy R/O CA

  27. Diagnostic Tests • Schilling test: Patient given Injection to saturate liver • Radioactive B12 orally • 24 hour urine collection • Impaired absorption radioactive B12 will not be excreted in urine

  28. Treatment • Prognosis good if treatment started • Up to 18 month for improvement • Dietary replacement if absorption normal • Red meat, liver, fish, poultry, shellfish, milk, dairy products, eggs, some fortified foods

  29. Treatment • If absorption abnormal Cynocobalamine IM 200ug/d X 2 weeks, Biweekly for 2 weeks, & monthly for life • Stop smoking, maintain ideal weight, teaching for med. Action, side effects, daily skin care, mouth & foot care, assess for breakdown, alt. Rest & activity, promote safe environment, avoid heat pad etc.

  30. Folic Acid Deficiency • Vitamin B complex • Seen in alcoholism, malabsorption syndromes, & pregnancy • Most prevalent in infants, adolescents, pregnant & lactating females, alcoholics & elderly • Increase incidence of drugs use during pregnancy

  31. Food Sources • Found in asparagus spears, beef liver, broccoli, collards, mushrooms, oatmeal, peanut butter, red beans, wheat germ

  32. Clinical Manifestations • Develop slowly over a period of months • Symptoms related to tissue hypoxia • Glossitis • Jaundice • Spleenomegaly

  33. Treatment • Administer folic acid every day until deficiency is corrected • High dises to patients with malabsorption problems • Folvite ; adults 250 to 1,000 mcg/d until hematological responses increases • Maintainance 400 mcg/day X 2