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