Abstract
The observation of increasing myelofibrosis has been interpreted as a sign of progression in the course of low risk patients with myelodysplastic syndrome (MDS). We report here a 64 year old, Caucasian woman who was diagnosed to have refractory anemia in 5/19/03 with a bone marrow findings showing hypercellularity of 95%, erythroid dysplastic changes with 1% myeloblasts with no ringed sideroblasts. Her cytogenetic study show normal chromosomes and she had an IPSS score of 0. However, she began to require red cell transfusions with no response to EPO and G-CSF therapy for 5 months when she was entered into an open label, multi-center clinical trial using Revlimid at 10 mg/day given orally in tablet form. Her growth factors were discontinued 4 weeks before entry and a baseline bone marrow study on 10/7/04 show a 90% cellular marrow with same abnormalities. In addition, she now has developed a grade III/IV diffuse reticulin fibers as shown by silver stains indicating increased myelofibrosis. She tolerated the treatment well with very little side effects and by second month of therapy, she had her last red cell transfusion and her hemoglobin level started to increase from 8.3 to 9.7 g/dl. Other than a mild neutropenia with ANC between 800 to 900/μL, she tolerated treatment well. She attained a normal hemoglobin of 12 g/dl at the 4th month. Repeat BM done at this time shows a normal cellularity of 50% but erythroid cells show persistence of dysplasia but overall fibrosis has reversed to Grade I/IV. She currently remains in remission 21 months on Revlimid treatment. Demonstration of reversal of myelofibrosis in MDS indicate that this drug may be effective in primary myelofibrosis and agnogenic myeloid metaplasia.
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