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

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

5. Leukostasis
Leukocytosis
Hyperleukocytosis
Leukostasis

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

13. Treatment
Hydration
Blood letting
Plasmapheresis

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