guidebook. - Fanconi Anemia Research Fund

Chapter 3: Treatment of Hematologic Abnormalities in FA
73
Transfused platelets should be leuko-depleted and
irradiated.
Amicar (epsilon aminocaproic acid) may be used as
an adjunct to platelet transfusion in the patient with
mucosal bleeding. The drug is given at a dose of
50-100 mg/kg every six hours, with a maximum dose of
around 12 grams/day. A loading dose of 200 mg/kg may
be considered. Amicar is usually administered for several
days until the clot is stabilized. Amicar is generally
contraindicated in patients with hematuria.
Additional factors that increase bleeding risk should be
minimized. Drugs that inhibit platelet function, such as
aspirin or non-steroidal anti-inflammatory drugs (e.g.,
ibuprofen), should be avoided. A soft toothbrush should
be used. Stool softeners should be administered if constipation
poses a risk of GI mucosal trauma. Activities
carrying a high risk of significant trauma (particularly
to the head or trunk) should be avoided.
Neutropenia
Patients with mild neutropenia are often asymptomatic.
Treatment with G-CSF or GM-CSF as described earlier
may be considered if the patient is having
neutropenia-related infectious complications with neutrophil
counts