Other

Question 1

What is the definition of aplastic anemia?

Answer 1

Peripheral blood showing Hb < 100g/L, platelets < 50,000 and granulocytes < 1.5, with a bone marrow showing decreasing cellularity in the absence of malignant
infiltration or fibrosis

Question 2

What is the definition of severe aplastic anemia?

Answer 2

Peripheral blood criteria (2/3):
- ANC < 0.5
- Retics < 40 
- Platelet < 20,000
Bone marrow criteria (1/2)
- Cellularity < 25%
- Cellularity 25-50% and < 30% hematopoietic cells

Question 3

What is the first line therapy for idiopathic SAA?

Answer 3

HSCT if matched sibling donor available;

Immunosuppressive therapy if no matched sibling donor available

Question 4

What constitutes immunosuppressive therapy for SAA?

Answer 4

• Cyclosporine 12 mg/kg/d
• ATG (equine) daily * 4 days; rabbit can be used in 2nd course to reduce risk of sensitization
• Prednisone, to avoid serum sickness; 2 mg/kg/d * 1 week with tapering over 3 weeks

Question 5

What is the clinical presentation of serum sickness following ATG (7 symptoms)?

Answer 5

1-2 weeks following ATG

• Fever
• Malaise
• Cutaneous eruptions (mostly urticaria)
• Arthritis/arthralgias
• GI Sx: No/Vo/Do
• Headaches, blurred vision
• Dyspnea, wheezing

Question 6

What is the treatment of serum sickness following ATG?

Answer 6

Prednisone 1 mg/kg/dose q 12h
Diphenhydramine
Clinical revory usually takes 1-4 weeks

Question 7

What is the treatment of congenital methemoglobinemia?

Answer 7

Ascorbic acid or methylene blue; only given for cosmetic reasons as patients are usually not or mildly symptomatic

Question 8

What is the treatment of acquired (acute) methemoglobinemia?

Answer 8

• *MEDICAL EMERGENCY
• Stop offending agent
• Methylene blue; usually decrease levels in 1-6 h
• For severe cases: transfusion (simple or exchange), hyperbaric chamber

Question 9

Name 5 symptoms of acute methemoglobinemia?

Answer 9

• Headache
• Lightheadness
• Tachycardia
• Dyspnea
• Alteration of consciousness, seizures
• Can lead to death

Question 10

Which infections are associated with splenomegaly?

Answer 10

• Viral infections: EBV, CMV, HIV
• Bacterial endocarditis
• Malaria, i.e. tropical splenomegaly
• Leishmaniasis
• Toxoplasmosis