Hematologic emergency
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This document discusses various hematologic emergencies and urgencies including leukostasis, hyperviscosity syndrome, thrombotic thrombocytopenic purpura, acute hemolytic transfusion reaction, cord compression, febrile neutropenia, bleeding diathesis, acute promyelocytic leukemia, and acute disseminated intravascular coagulation. It provides details on symptoms, diagnostic criteria, and treatment approaches for each condition. The goal is to identify life-threatening hematologic emergencies, determine their causes, and initiate appropriate urgent medical management.
Hematologic emergency
- 1. Hematologic Emergency Kaipol Takpradit, M.D.
- 2. How emergency? Life threatening? Organ threatening?
- 3. Hematologic emergency Leukostasis Hyperviscosity syndrome Thrombotic Thrombocytopenic Purpura Acute Hemolytic Transfusion Reaction Cord compression
- 4. Hematologic urgency Febrile neutropenia Bleeding diathesis Other transfusion reaction AML M3 or APL Acute DIC
- 6. Leukostasis Depend on number and type Blast cells Mature cells Myeloid Lymphoid
- 7. Symptoms CNS: headache, confusion, tinnitus, papilledema, ataxia, stupor, obtundation, hemorrhage Lungs: dyspnea, hypoxemia Others
- 8. Treatment Leukapheresis Chemotherapy Hydroxyurea 50-100 mkd or 3g/m2 Ara-c 100 mg/m2 Steroid Definite induction therapy
- 9. Treatment Hydration Prevention of tumor lysis syndrome Avoid cellular blood component Platelet transfusion is allowed Not applicable to AML M3
- 10. Hyperviscosity Result from cellular and/or serum component Serum viscosity normally is ≤ 1.8 centipoise (cP) Most symptomatic cases have serum viscosity of 6-7 cP
- 11. Hyperviscosity Cellular component Polycythemia vera Hyperleukocytosis Serum component Paraproteinemia Cryoglobulinemia
- 12. Hyperviscosity Bleeding, particularly from the oronasal areas Purpura VA drop, retinopathy Neurologic symptoms Dyspnea, expanded plasma volume, and CHF
- 14. TTP Abnormally large von Willebrand factor Result in “white” platelet thrombi Disseminated tissue ischemia
- 15. TTP pentad Fever Thrombocytopenia MAHA blood picture Fluctuating neurological symptom Renal failure
- 16. TTP PBS Decreased ADAMTS-13 to < 10% von Willebrand factor multimer assay
- 17. Treatment Plasma exchange Plasma infusion Immunosuppressive Look for cause HIV Thienopyridine
- 18. TTP/HUS Difficulty to distinguish these two condition TTP predominantly occur in adult with neurological symptoms HUS predominantly occur in children with renal complication
- 19. Acute hemolytic transfusion reaction From ABO mismatch (IgM mediated) Intravascular hemolysis by complement Inflammatory cytokine (bradykinin, etc) can cause hemodynamic instability Renal and metabolic complication must be aware
- 20. Treatment Close observe for reaction after transfusion commenced Immediately stop transfusion in case of reaction At least 30 ml of ABO incompatible blood can be fatal
- 21. Management Most incidences are clerkish error Must identify cause(s) to prevent further incidence
- 22. Other transfusion reaction Delayed hemolytic transfusion reaction Febrile non-hemolytic transfusion reaction Post-transfusion purpura Bacterial contamination
- 23. Management With hold transfusion Assess vital sign Take culture from transfusion bag and patient Send the bag to blood bank for re-cross match
- 24. Cord compression Organ threatening event Must be managed in time
- 25. Cause of cord compression Tumor mass Malignant tumor Extramedullary hematopoiesis Other organ Fracture lytic bone Other cause
- 26. Investigation MRI spine to determine cause of cord compression Tumor mass Bony structure Leptomeningeal infiltration
- 27. Treatment Radiation and transfusion for extramedullary hematopoietic mass Steroid for lymphoma or plasmacytoma Dexamethasone 100 mg bolus then 24 mg q 6 hr for 3 days then tape off Surgery for boney compression
- 28. Febrile neutropenia Definition Neutropenia: ANC < 500/µL or < 1,000/µL and decreasing to < 500/µL in 48hr Fever: Temp. ≥ 38.3°c (orally) or ≥ 38°c over 1 hr
- 29. Management History and physical exam Look for tender site Pay attention on oral and perianal area PR is contraindicated Gram stain from body fluid Empiric antibiotic Promptly reevaluate for fungus if fever persist
- 30. Empirical antibiotic Pseudomonas coverage Risk of resistant bacteria Cefepime, piperacillin-tazobactam Imipenem-cilastatin, meropemen Vancomycin may be added for catherter- related, skin and soft tissue infection, pneumonia, or hemodynamic instability
- 31. Empirical antibiotic Patient with low risk may treat as OPD case Amoxi/Clavulanate (1g) bid Ciprofloxacin (500mg) bid Follow up in a few days for H/C result
- 32. G-CSF No survival benefit from G-CSF May reduce neutropenic duration Generally not recommended
- 33. Bleeding diathesis Minor - not significant Major - significant Fatal bleeding Bleeding that symptomatic in critical site Fall in Hb of ≥ 2g/dL or leading to transfusion of ≥ 2 unit of PRC or whole blood
- 34. Management Identify cause Primary hemostasis Secondary hemostasis
- 35. Management Replacement therapy for deficient component For severe-hemophiliac patient replacement is needed if has history of trauma Don’t wait for clinical bleeding
- 36. Management Anticoagulant bleeding Heparin Warfarin Antiplatelet Aspirin Other antiplatelet (thienopyridine, etc.)
- 37. Heparin Protamine sulfate 1 mg for 100 unit of heparin For quick calculation -> rate heparin x 2 For LMWH protamine can neutralize about 60% for administered doses No specific antidote for pentasaccharide and heparinoid
- 38. Warfarin Usual target INR 2-3 INR 3-5 reduce dose INR 5-9 hold 1 - 2 days and reduce dose Or oral Vit.K 1-2.5 mg and reduce dose INR >9 oral Vit.K 2.5-5 mg and reduce dose If major bleeding Vit.k 10 mg IV and FFP
- 39. Warfarin Food and drug interaction Laxative or diarrhea can alter intestinal bacteria population and reduce Vit.K Minimally elevated INR with precipitating event need no dose adjustment
- 40. Antiplatelet Cause platelet dysfunction Hold ASA 5-7 days Hold thienopyridine 7-10 days For high risk for thrombosis may continue ASA but still have to hold thienopyridine
- 41. Other transfusion reaction Delayed hemolytic transfusion reaction Febrile non-hemolytic transfusion reaction Posttransfusion purpura TRALI Bacterial comtamination
- 42. Acute promyelocytic leukemia Very high early mortality Very good long-term prognosis Usually cause DIC at presentation DIC exaggerated by induction chemotherapy Differentiating agent combined with chemotherapy is preferable (ATRA, ATO)
- 43. Acute Disseminated Intravascular Coagulation Can occur in setting of large tissue injury Trauma, infection, etc Consumptive coagulopathy increased fibrin degradation product (FDP, D- dimer) Correct cause is definite treatment
- 44. Acute Disseminated Intravascular Coagulation Rarely cause bleeding Transfusion is only needed in bleeding patients Transfusion to keep platelet > 20,000/µL and fibrinogen > 100 mg/dL in bleeding patients
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