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

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

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

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

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

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

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

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

Sickle Cell Anemia Complications Arthropathy Aseptic necrosis Hemarthosis Septic arthritis Priapism

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

Sickle Cell Anemia Management Oxygen IV fluids Analgesia

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Leukemia Management Oxygen IV Analgesia Infection control procedures