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Hematologic Emergencies

Learn about the pathophysiology, incidence, complications, and management of sickle cell anemia, hemophilia, and disseminated intravascular coagulation. Understand the causes, signs, symptoms, and treatment protocols for these critical hematologic conditions. Discover essential information on leukemia, including its definition, clinical manifestations, and management strategies.

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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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