HEME/ONC/ENDO part 3

Question 1

MYELODYSPLASTIC SYNDROME

TREATMENT FOR AA AND MDS 
• Blood product support
o _____ transfusions
o _____ transfusions

•	Blood cell growth factors
o	Epoetin alpha (Procrit)
o	Darbepoetin alpha (Aranesp)
o	Filgrastim (Neupogen)
o	Tbo-filgastim (Granix)
o	Eltrombopag (Promacta)
o	Romiplostim (Nplate)

• _____ transplant
• Hypomethylating agents in low-risk MDS
o Azacitidine (Vidaza)
o Decitabine (Dacogen

Answer 1

PRBC
platelet
Stem cell

Question 2

IMMUNE THROMBOCYTOPENIA

• All patients should be referred to hematology
• Admit to hospital if severe thrombocytopenia and/or high risk for bleeding
• Mainstay of treatment is _____
• Add _____ (intravenous immunoglobulin)
• When both given, response is seen in 24 to 36 hrs
• Avoid platelet transfusion unless active bleeding
• Rituximab is used for corticosteroid-refractory ITP
• Promacta (oral) and Nplate (SC) can also be used
• _____ has a response rate of over 50% and is used in refractory ITP
o Main serious risks are hypersensitivity reaction and acute renal failure

Answer 2

corticosteroids
IVIG
Splenectomy

Question 3

HEPARIN INDUCED THROMBOCYTOPENIA

TREATMENT 
• Treatment should begin if HIT strongly suspected
o Even before lab results are available
o Patient with progressive thrombocytopenia and new thrombosis
• Immediately STOP all _____ products
• Add heparin to allergy list
• If thrombosis not already found, duplex Doppler ultrasound BLE to rule out subclinical DVT
• Avoid platelet transfusions
• Start alternative anticoagulant, typically a direct thrombin inhibitor such as _____ or selective factor Xa inhibitor such as _____ in hospital

Answer 3

heparin
argatroban
fondaparinux

Question 4

DISSEMINATED INTRAVASCULAR COAGULATION

TREATMENT 
• Most important: treat underlying cause
o _____
o Chemotherapy
o Delivery of conceptus
• Establish baseline platelet count, PT, PTT, D-dimer, and _____
• Transfuse blood products if significant bleeding
• Give _____ to maintain fibrinogen > 100
• Give ___ with goal PT and PTT < 1.5x normal
• Follow and trend labs closely

Answer 4

antimicrobials
fibrinogen
cryoprecipitate
FFP

Question 5

HEMOPHILIA A AND B

TREATMENT 
• Mainstay of treatment is plasma-derived or recombinant factor ___ or ___ products
• Primary prophylaxis with serial infusions to prevent bleeding
• Also episodic treatment during surgery, prior to high-risk activities, or for active bleeding
• Patients with mild hemophilia A may respond to as-needed IV or intranasal _____
• Gene therapy is now here and will change the treatment landscape over the next decade
• Factor inhibitors are a major clinical problem for patients with hemophilia
o Hemophilia A: inhibitors to factor VIII will develop in 20-25% of patients
o Hemophilia B: inhibitors to factor XI will develop in less than 5% of patients
• All patients should be followed in a hemophilia center
• When to admit to hospital:
o Major invasive procedures
o Bleeding that is unresponsive to outpatient treatment

Answer 5

VIII
IX
DDAVP