Abstract
We report on a patient with Fanconi's anemia (FA) who developed a myelodysplastic syndrome (RAEB-T) with complex karyotypic abnormalities (trp 1q23 q 42, monosomy 20, trisomy 13) at the age of 28. The patient achieved a complete hematological and cytogenetic remission after treatment with sequential high-dose cytosine arabinoside/mitoxantrone followed by G-CSF (5 μg/kg). Bone marrow hypoplasia was prolonged with 38 days of granulocytopenia <500/μl and 62 days of platelet transfusion dependency. Nonhematological toxicity did not exceed that of patients without underlying FA. Remission duration was 7 months. This observation shows the feasibility of high-dose Ara C treatment in patients with FA and MDS. Although hematopoiesis remained clonal in remission, the suppression of the cytogenetically abnormal clones transiently reversed the antecedent long-lasting pancytopenia.
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Received: 16 September 1996 / Accepted: 27 January 1997
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Verbeek, W., Haase, D., Schoch, C. et al. Induction of a hematological and cytogenetic remission in a patient with a myelodysplastic syndrome secondary to Fanconi's anemia employing the S-HAM regimen. Ann Hematol 74, 275–277 (1997). https://doi.org/10.1007/s002770050299
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DOI: https://doi.org/10.1007/s002770050299