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Chapter 9: Matched Sibling Donor HSCT 185 should be considered in the final equation. If the patient has acceptable counts and is generally healthy on no medications, including androgens, transplant can probably be safely delayed. Evidence of a clone, MDS or leukemia Patients with FA may develop cytogenetic clones. These clones may disappear or be replaced by some other clone on a subsequent bone marrow test done just a few months later. The danger signs which should lead to transplantation include a clone which is steadily increasing in percentage, or a clone involving chromosome 7 or showing a gain in the 3q26q29 segment. Data suggest that such patients have a higher risk of progression to MDS or AML. Myelodysplasia is a hard call in aplastic-appearing marrows of FA patients. Mild dysplasia is often seen, but significant multilineage dysplasia should prompt consideration for transplant. It is advisable to have bone marrow smears reviewed by physicians at a center with extensive experience in FA patients. Patients with FA who have developed advanced MDS or leukemia clearly need immediate referral for transplant. The goal is to proceed to transplant before definite advanced MDS or leukemia develops. Occasionally, patients do progress quickly into advanced MDS or leukemia, making it necessary to transplant them at once. This is a very difficult situation which is best left in the hands of a center with extensive experience in FA. Some centers use an induction protocol prior to transplant, with a regimen specifically modified for FA patients. Patients are first administered a mild course of chemotherapy to get them into remission. Two to three weeks later, patients begin preparative therapy for a bone marrow transplant. Other centers
186 <strong>Fanconi</strong> <strong>Anemia</strong>: Guidelines for Diagnosis and Management proceed directly to transplant using a total body irradiation or busulfan based regimen. Organ Function Parameters Patients should have adequate renal function (GFR >50 ml/min/1.73 m 2 ), cardiac function (shortening fraction >27%), and liver function (bilirubin
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Chapter 4 Gastrointestinal, Hepatic
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Chapter 12 Novel Treatment Options
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Chapter 13 Head and Neck Squamous C
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Chapter 14 The Adult Fanconi Anemia
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