Hematologic Emergencies

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Hematologic Emergencies
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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

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Sickle Cell Anemia

• Pathophysiology
• Sickled RBCs cannot pass through small vessels
• Thrombosis
• Infarction
• Sickled RBCs are fragile
• Hemolysis

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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

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Sickle Cell Anemia

• Incidence
• Also occurs in populations from
• Puerto Rico
• Turkey
• India
• Middle East
• Southern Europe
• North Africa

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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

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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

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Sickle Cell Anemia

• Hemolytic Crisis
• Rupture of sickled cells
• Fall in hematocrit with jaundice

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Sickle Cell Anemia

• Aplastic Crisis
• Suppression of RBC production
• Life threatening decrease in hematocrit

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Sickle Cell Anemia

• Complications
• Acute Respiratory Insufficiency
• Thrombus formation
• Pneumonia
• CNS Involvement
• Vascular occlusion
• TIA, CVA

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Sickle Cell Anemia

• Complications
• Arthropathy
• Aseptic necrosis
• Hemarthosis
• Septic arthritis
• Priapism

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Sickle Cell Anemia

• Complications
• Renal infarction
• Flank pain
• Hematuria
• Narcotic drug addiction

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Sickle Cell Anemia

• Management
• Oxygen
• IV fluids
• Analgesia

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Hemophilia

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

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Hemophilia

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

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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

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Disseminated Intravascular Coagulation (DIC)

• Pathophysiology
• Damage tissue, blood vessels, blood cells
• Clotting mechanism activation
• Excess clotting
• Diffuse microthrombi
• Consumption of clotting factors

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DIC

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

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DIC

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

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DIC

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

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DIC

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

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DIC

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

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DIC

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

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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

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Leukemia

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

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Leukemia

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

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Leukemia

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

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Leukemia

• Management
• Oxygen
• IV
• Analgesia
• Infection control procedures