guidebook. - Fanconi Anemia Research Fund

Chapter 3: Treatment of Hematologic Abnormalities in FA
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but a lower hematocrit trough, or more frequent visits
to maintain a higher hemoglobin—is a decision to be
made by the patient, the patient’s family, and the treating
hematologist.
All patients should receive red blood cells that have
been leuko-depleted. The most widely used and effective
method is to use a leuko-depletion filter. Irradiated
blood products should be used to avoid transfusionassociated
graft-versus-host disease. Some centers use
only CMV-negative red blood cells, while others accept
leuko-depletion as an alternative to CMV-negative
products. Extended antigen matching may be important
for patients in certain racial groups, where minor
antigen mismatch is more commonly encountered.
Directed donation is not encouraged, especially for
family members. The use of family members as directed
donors may cause alloimmunization to an antigen that
would increase the risk of graft rejection after sibling
donor hematopoietic stem cell transplant.
Secondary iron overload
Each mL of transfused packed red cells contains
approximately 0.7 mg of iron. Since the human body
lacks mechanisms to actively eliminate excess iron,
patients who receive multiple red blood cell transfusions
are at risk for accumulating toxic levels of iron
overload. The liver is a primary site of iron accumulation,
and hepatic fibrosis and cirrhosis may result. Iron
deposition in the myocardium may cause dysrhythmias
and cardiac failure. Cardiac decompensation may
be sudden and acute despite regular monitoring with
electrocardiograms and measurements of cardiac function.
Recent data in the thalassemia population suggests
that T2* MRI may be a better modality to follow
cardiac function and cardiac siderosis in patients with