Internal Medicine

P1: SBT

0521779407-04 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:8

152 Aplastic Anemia

3) High-dose cyclophosphamide (50 mg/kg/d×4 days)

■Advantages:

➣Restores normal hematopoiesis (curative potential): Low risk of

relapse. Best results (∼70% cure rate) if used as initial therapy;

lower response rate in patients previously treated with ATG/CSA

➣Does not require a stem cell donor

➣No risk of GVHD

➣Few age restrictions, available to virtually all patients with SAA

■Disadvantages:

➣Slow hematopoietic improvement

➣Intensive supportive care required during first 2 months

follow-up

■Regularly assess potential complications of disease and therapy.

■bone marrow transplantation: daily blood counts

➣monitor for infections/bleeding

➣monitor for GVHD

■ATG/CSA: daily blood counts

➣monitor for infections/bleeding

➣allergic reactions to ATG

Monitor for development of PNH or MDS

■high-dose cyclophosphamide: daily blood counts

➣monitor for infections/bleeding

Monitor for development of PNH or MDS

Routine

■bone marrow aspirate, biopsy and cytogenetics are discretionary.

complications and prognosis

■ATG/CSA:

➣serum sickness

hyperglycemia

PNH (10% to 30%)

MDS (10% to 20%)

relapse (20% to 40%)

aseptic necrosis (5% to 10%)

■bone marrow transplantation GVHD (acute and chronic)

➣second cancers (rare)

Prognosis

■1 year mortality > than 70% with supportive care alone

■mortality following therapy is greatest in the first three months