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Hematologic Emergency     Kaipol Takpradit, M.D.
How emergency?Life threatening?Organ threatening?
Hematologic emergency Leukostasis Hyperviscosity syndrome Thrombotic Thrombocytopenic Purpura Acute Hemolytic Transfusion ...
Hematologic urgencyFebrile neutropeniaBleeding diathesisOther transfusion reactionAML M3 or APLAcute DIC
LeukostasisLeukocytosisHyperleukocytosisLeukostasis
LeukostasisDepend on number and type  Blast cells  Mature cells  Myeloid  Lymphoid
SymptomsCNS: headache, confusion, tinnitus,papilledema, ataxia, stupor, obtundation,hemorrhageLungs: dyspnea, hypoxemiaOth...
TreatmentLeukapheresisChemotherapy  Hydroxyurea 50-100 mkd or 3g/m2  Ara-c 100 mg/m2  Steroid  Definite induction therapy
TreatmentHydrationPrevention of tumor lysis syndromeAvoid cellular blood componentPlatelet transfusion is allowedNot appli...
HyperviscosityResult from cellular and/or serum componentSerum viscosity normally is ≤ 1.8 centipoise(cP)Most symptomatic ...
HyperviscosityCellular component  Polycythemia vera  HyperleukocytosisSerum component  Paraproteinemia  Cryoglobulinemia
HyperviscosityBleeding, particularly from the oronasalareasPurpuraVA drop, retinopathyNeurologic symptomsDyspnea, expanded...
TreatmentHydrationBlood lettingPlasmapheresis
TTPAbnormally large von Willebrand factorResult in “white” platelet thrombiDisseminated tissue ischemia
TTP pentadFeverThrombocytopeniaMAHA blood pictureFluctuating neurological symptomRenal failure
TTPPBSDecreased ADAMTS-13 to < 10%von Willebrand factor multimer assay
TreatmentPlasma exchangePlasma infusionImmunosuppressiveLook for cause  HIV  Thienopyridine
TTP/HUSDifficulty to distinguish these two conditionTTP predominantly occur in adult withneurological symptomsHUS predomina...
Acute hemolytictransfusion reactionFrom ABO mismatch (IgM mediated)Intravascular hemolysis by complementInflammatory cytoki...
TreatmentClose observe for reaction after transfusioncommencedImmediately stop transfusion in case ofreactionAt least 30 m...
ManagementMost incidences are clerkish errorMust identify cause(s) to prevent furtherincidence
Other transfusion      reactionDelayed hemolytic transfusion reactionFebrile non-hemolytic transfusion reactionPost-transf...
ManagementWith hold transfusionAssess vital signTake culture from transfusion bag andpatientSend the bag to blood bank for...
Cord compressionOrgan threatening eventMust be managed in time
Cause of cord       compressionTumor mass  Malignant tumor  Extramedullary hematopoiesisOther organ  Fracture lytic bone  ...
InvestigationMRI spine to determine cause of cordcompression  Tumor mass  Bony structure  Leptomeningeal infiltration
TreatmentRadiation and transfusion for extramedullaryhematopoietic massSteroid for lymphoma or plasmacytoma  Dexamethasone...
Febrile neutropeniaDefinition  Neutropenia: ANC < 500/µL or < 1,000/µL  and decreasing to < 500/µL in 48hr  Fever: Temp. ≥ ...
ManagementHistory and physical exam  Look for tender site  Pay attention on oral and perianal area  PR is contraindicatedG...
Empirical antibioticPseudomonas coverageRisk of resistant bacteria  Cefepime, piperacillin-tazobactam  Imipenem-cilastatin...
Empirical antibioticPatient with low risk may treat as OPD case  Amoxi/Clavulanate (1g) bid  Ciprofloxacin (500mg) bidFollo...
G-CSFNo survival benefit from G-CSFMay reduce neutropenic durationGenerally not recommended
Bleeding diathesisMinor - not significantMajor - significant  Fatal bleeding  Bleeding that symptomatic in critical site  Fa...
ManagementIdentify cause  Primary hemostasis  Secondary hemostasis
ManagementReplacement therapy for deficient componentFor severe-hemophiliac patient replacementis needed if has history of ...
ManagementAnticoagulant bleeding  Heparin  WarfarinAntiplatelet  Aspirin  Other antiplatelet (thienopyridine, etc.)
HeparinProtamine sulfate  1 mg for 100 unit of heparin  For quick calculation -> rate heparin x 2For LMWH protamine can ne...
WarfarinUsual target INR 2-3INR 3-5 reduce doseINR 5-9 hold 1 - 2 days and reduce dose  Or oral Vit.K 1-2.5 mg and reduce ...
WarfarinFood and drug interactionLaxative or diarrhea can alter intestinalbacteria population and reduce Vit.KMinimally el...
AntiplateletCause platelet dysfunctionHold ASA 5-7 daysHold thienopyridine 7-10 daysFor high risk for thrombosis may conti...
Other transfusion      reactionDelayed hemolytic transfusion reactionFebrile non-hemolytic transfusion reactionPosttransfu...
Acute promyelocytic     leukemiaVery high early mortalityVery good long-term prognosisUsually cause DIC at presentationDIC...
Acute DisseminatedIntravascular Coagulation  Can occur in setting of large tissue injury    Trauma, infection, etc  Consum...
Acute DisseminatedIntravascular Coagulation  Rarely cause bleeding  Transfusion is only needed in bleeding  patients  Tran...
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Hematologic emergency

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Brief introduction to hematologic emergency and urgency. Designed for 5th year medical student.

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Hematologic emergency

  1. 1. Hematologic Emergency Kaipol Takpradit, M.D.
  2. 2. How emergency?Life threatening?Organ threatening?
  3. 3. Hematologic emergency Leukostasis Hyperviscosity syndrome Thrombotic Thrombocytopenic Purpura Acute Hemolytic Transfusion Reaction Cord compression
  4. 4. Hematologic urgencyFebrile neutropeniaBleeding diathesisOther transfusion reactionAML M3 or APLAcute DIC
  5. 5. LeukostasisLeukocytosisHyperleukocytosisLeukostasis
  6. 6. LeukostasisDepend on number and type Blast cells Mature cells Myeloid Lymphoid
  7. 7. SymptomsCNS: headache, confusion, tinnitus,papilledema, ataxia, stupor, obtundation,hemorrhageLungs: dyspnea, hypoxemiaOthers
  8. 8. TreatmentLeukapheresisChemotherapy Hydroxyurea 50-100 mkd or 3g/m2 Ara-c 100 mg/m2 Steroid Definite induction therapy
  9. 9. TreatmentHydrationPrevention of tumor lysis syndromeAvoid cellular blood componentPlatelet transfusion is allowedNot applicable to AML M3
  10. 10. HyperviscosityResult from cellular and/or serum componentSerum viscosity normally is ≤ 1.8 centipoise(cP)Most symptomatic cases have serum viscosityof 6-7 cP
  11. 11. HyperviscosityCellular component Polycythemia vera HyperleukocytosisSerum component Paraproteinemia Cryoglobulinemia
  12. 12. HyperviscosityBleeding, particularly from the oronasalareasPurpuraVA drop, retinopathyNeurologic symptomsDyspnea, expanded plasma volume, and CHF
  13. 13. TreatmentHydrationBlood lettingPlasmapheresis
  14. 14. TTPAbnormally large von Willebrand factorResult in “white” platelet thrombiDisseminated tissue ischemia
  15. 15. TTP pentadFeverThrombocytopeniaMAHA blood pictureFluctuating neurological symptomRenal failure
  16. 16. TTPPBSDecreased ADAMTS-13 to < 10%von Willebrand factor multimer assay
  17. 17. TreatmentPlasma exchangePlasma infusionImmunosuppressiveLook for cause HIV Thienopyridine
  18. 18. TTP/HUSDifficulty to distinguish these two conditionTTP predominantly occur in adult withneurological symptomsHUS predominantly occur in children withrenal complication
  19. 19. Acute hemolytictransfusion reactionFrom ABO mismatch (IgM mediated)Intravascular hemolysis by complementInflammatory cytokine (bradykinin, etc) cancause hemodynamic instabilityRenal and metabolic complication must beaware
  20. 20. TreatmentClose observe for reaction after transfusioncommencedImmediately stop transfusion in case ofreactionAt least 30 ml of ABO incompatible blood canbe fatal
  21. 21. ManagementMost incidences are clerkish errorMust identify cause(s) to prevent furtherincidence
  22. 22. Other transfusion reactionDelayed hemolytic transfusion reactionFebrile non-hemolytic transfusion reactionPost-transfusion purpuraBacterial contamination
  23. 23. ManagementWith hold transfusionAssess vital signTake culture from transfusion bag andpatientSend the bag to blood bank for re-crossmatch
  24. 24. Cord compressionOrgan threatening eventMust be managed in time
  25. 25. Cause of cord compressionTumor mass Malignant tumor Extramedullary hematopoiesisOther organ Fracture lytic bone Other cause
  26. 26. InvestigationMRI spine to determine cause of cordcompression Tumor mass Bony structure Leptomeningeal infiltration
  27. 27. TreatmentRadiation and transfusion for extramedullaryhematopoietic massSteroid for lymphoma or plasmacytoma Dexamethasone 100 mg bolus then 24 mg q 6 hr for 3 days then tape offSurgery for boney compression
  28. 28. Febrile neutropeniaDefinition 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. 29. ManagementHistory and physical exam Look for tender site Pay attention on oral and perianal area PR is contraindicatedGram stain from body fluidEmpiric antibioticPromptly reevaluate for fungus if fever persist
  30. 30. Empirical antibioticPseudomonas coverageRisk 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. 31. Empirical antibioticPatient with low risk may treat as OPD case Amoxi/Clavulanate (1g) bid Ciprofloxacin (500mg) bidFollow up in a few days for H/C result
  32. 32. G-CSFNo survival benefit from G-CSFMay reduce neutropenic durationGenerally not recommended
  33. 33. Bleeding diathesisMinor - not significantMajor - 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. 34. ManagementIdentify cause Primary hemostasis Secondary hemostasis
  35. 35. ManagementReplacement therapy for deficient componentFor severe-hemophiliac patient replacementis needed if has history of trauma Don’t wait for clinical bleeding
  36. 36. ManagementAnticoagulant bleeding Heparin WarfarinAntiplatelet Aspirin Other antiplatelet (thienopyridine, etc.)
  37. 37. HeparinProtamine sulfate 1 mg for 100 unit of heparin For quick calculation -> rate heparin x 2For LMWH protamine can neutralize about60% for administered dosesNo specific antidote for pentasaccharide andheparinoid
  38. 38. WarfarinUsual target INR 2-3INR 3-5 reduce doseINR 5-9 hold 1 - 2 days and reduce dose Or oral Vit.K 1-2.5 mg and reduce doseINR >9 oral Vit.K 2.5-5 mg and reduce doseIf major bleeding Vit.k 10 mg IV and FFP
  39. 39. WarfarinFood and drug interactionLaxative or diarrhea can alter intestinalbacteria population and reduce Vit.KMinimally elevated INR with precipitatingevent need no dose adjustment
  40. 40. AntiplateletCause platelet dysfunctionHold ASA 5-7 daysHold thienopyridine 7-10 daysFor high risk for thrombosis may continueASA but still have to hold thienopyridine
  41. 41. Other transfusion reactionDelayed hemolytic transfusion reactionFebrile non-hemolytic transfusion reactionPosttransfusion purpuraTRALIBacterial comtamination
  42. 42. Acute promyelocytic leukemiaVery high early mortalityVery good long-term prognosisUsually cause DIC at presentationDIC exaggerated by induction chemotherapyDifferentiating agent combined withchemotherapy is preferable (ATRA, ATO)
  43. 43. Acute DisseminatedIntravascular 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. 44. Acute DisseminatedIntravascular 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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