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Hematologic Emergencies

Hematologic Emergencies. Sickle Cell Anemia. Pathophysiology Genetic abnormality causes red cells to contain hemoglobin S rather than hemoblobin A At low oxygen tensions hemoglobin S forms long, rigid rods RBCs become distorted, sickle-shaped. Sickle Cell Anemia. Pathophysiology

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Hematologic Emergencies

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  1. Hematologic Emergencies

  2. Sickle Cell Anemia • Pathophysiology • Genetic abnormality causes red cells to contain hemoglobin S rather than hemoblobin A • At low oxygen tensions hemoglobin S forms long, rigid rods • RBCs become distorted, sickle-shaped

  3. Sickle Cell Anemia • Pathophysiology • Sickled RBCs cannot pass through small vessels • Thrombosis • Infarction • Sickled RBCs are fragile • Hemolysis

  4. Sickle Cell Anemia • Incidence • Gene present in 10% of black population (sickle cell trait) • One in every 400 to 600 blacks has sickle cell anemia

  5. Sickle Cell Anemia • Incidence • Also occurs in populations from • Puerto Rico • Turkey • India • Middle East • Southern Europe • North Africa

  6. Sickle Cell Anemia • Incidence • Sickle cell gene survives by providing resistance to malaria • Two normal genes = malaria = death • Two sickle-cell genes = sickle cell anemia = death • One normal gene/one sickle-cell gene = survival • Persons with sickle cell trait survive, pass gene to offspring

  7. Sickle Cell Anemia • Thrombotic crisis (vasoocclusive-infarctive) • Precipitated by: • Cold exposure • High altitude • Dehydration • Infections • Gnawing pain in bones (tibias, back), joints, muscles, and abdomen

  8. Sickle Cell Anemia • Hemolytic Crisis • Rupture of sickled cells • Fall in hematocrit with jaundice

  9. Sickle Cell Anemia • Aplastic Crisis • Suppression of RBC production • Life threatening decrease in hematocrit

  10. Sickle Cell Anemia • Complications • Acute Respiratory Insufficiency • Thrombus formation • Pneumonia • CNS Involvement • Vascular occlusion • TIA, CVA

  11. Sickle Cell Anemia • Complications • Arthropathy • Aseptic necrosis • Hemarthosis • Septic arthritis • Priapism

  12. Sickle Cell Anemia • Complications • Renal infarction • Flank pain • Hematuria • Narcotic drug addiction

  13. Sickle Cell Anemia • Management • Oxygen • IV fluids • Analgesia

  14. Hemophilia • Cause • Inherited, sex linked recessive gene • Produces clotting factor deficiencies • Hemophilia A (Factor VIII) • Hemophilia B (Factor IX)

  15. Hemophilia • Signs • Swollen, painful joints • Local bleeding out of proportion to injury • Subcutaneous bleeding • Bleeding from mucous membranes • Abdominal pain, distension • Hematemesis, melena

  16. Hemophilia • Management • Standard techniques for hemorrhage/ hypovolemia • Apply ice packs to hemarthoses/ bleeding sites • In hospital, administration of • clotting factor • cryoprecipitate, or • fresh frozen plasma

  17. Disseminated Intravascular Coagulation (DIC) • Pathophysiology • Damage tissue, blood vessels, blood cells • Clotting mechanism activation • Excess clotting • Diffuse microthrombi • Consumption of clotting factors

  18. DIC • Pathophysiology • Split fibrin products from clots inhibit clotting • Clotting factor consumption, split fibrin products lead to: • Hemorrhage • Shock

  19. DIC • Causes • Gram negative sepsis • Abruptio placentae/missed abortions • Burns • Heat Stroke • Shock • Transfusion reaction with hemolysis

  20. DIC • Signs/Symptoms • Petechiae and ecchymosis • Bleeding from mucus membranes, orifices, venipuncture sites • Blood in urine, stool, vomitus, sputum • Signs/Symptoms of hypovolemia

  21. DIC All patients with predisposing conditions or who are critically ill should be monitored for signs of prolonged clotting or of bleeding

  22. DIC • Management • Control bleeding • Treat hypovolemia • Fresh frozen plasma or cryoprecipitate to inhibit bleeding, activate normal clotting • Identify and treat cause

  23. DIC • Management • Heparin to inhibit additional clotting (controversial) • Antifibrinolytic Agents (Amicar) to inhibit fibrinolysis and formation of split fibrin products (controversial)

  24. Anticoagulant Use with Hemorrhage • Control bleeding/treat hypovolemia with standard techniques • Use ice packs to supplement pressure dressings • Antidotes • Coumadin • Vitamin K, fresh frozen plasma • Heparin • Protamine sulfate

  25. Leukemia • Definition • Neoplastic disorder of blood cell forming tissues • Causes uncontrolled production of white cells

  26. Leukemia • Clinical manifestations • Decreased RBCs • Bleeding (decreased platelets) • Infection • WBC immaturity • Chemotherapy

  27. Leukemia • Clinical manifestations • Bone pain • Liver, spleen, lymph node enlargement • Anorexia

  28. Leukemia • Management • Oxygen • IV • Analgesia • Infection control procedures

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