guidebook. - Fanconi Anemia Research Fund

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Fanconi Anemia: Guidelines for Diagnosis and Management
and chronic GvHD after alternate donor HSCT, and
it appears to have improved disease-free survival in
patients with FA.
Regardless of the source of hematopoietic cells, most
patients receive cyclosporine A or tacrolimus for 6–12
months after HSCT to reduce the risk of GvHD. The
side effects of GvHD prevention strategies are shown in
Table 9.
Table 9: GvHD Prevention Therapy Side Effects
T-Cell Depletion
• Graft failure
• Slow immune recovery and infection
Cyclosporine A/Tacrolimus
• Poor kidney function or failure (dialysis)
• Blood chemistry imbalances (low potassium and
magnesium)
• Swelling of gums
• Excess body hair growth
• High blood pressure
• Bleeding problems
• Neurological side effects (seizures, coma, confusion,
tingling/burning sensations, involuntary shaking of
extremities)
• Infection
Methylprednisolone
• Infection
• Mood swings
• High blood sugar (requiring insulin)
• High blood pressure
• Avascular necrosis of long bones (damage to hip, knees,
and shoulder bones most commonly)
Regardless of the prophylactic approach used, GvHD
can still occur. The more severe the GvHD (e.g., grade
3-4 disease), the higher the risk of death, mostly due to
opportunistic infection. If GvHD occurs, the mainstay