Abstract
Thrombocytosis is not a frequent event in myelodysplasia (MDS) and is observed mainly in 5q- syndrome and MDS/myeloproliferative (MPD) overlap syndromes. The pathogenetic mechanism of thrombocytosis in 5q- has not been fully elucidated to-date. Bortezomib is a proteasome inhibitor which seems to be effective in MDS. We present here the first case in the literature with MDS/MPD syndrome, sole 5q- anomaly and thrombocytosis in which bortezomib administration normalized platelet count, produced a major erythroid response, and reduced levels of interleukin-6 (IL-6) and TNF-alpha while increased levels of IL-4 in the bone marrow plasma. The study of such cases will reveal the exact role of bortezomib in the management of MDS/MPD.
MeSH terms
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Antineoplastic Agents / therapeutic use*
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Boronic Acids / therapeutic use*
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Bortezomib
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Chromosomes, Human, Pair 5 / genetics*
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Female
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Humans
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Interleukin-4 / metabolism
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Interleukin-6 / metabolism
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Middle Aged
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Myelodysplastic Syndromes / complications
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Myelodysplastic Syndromes / drug therapy*
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Myelodysplastic Syndromes / genetics
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Myeloproliferative Disorders / complications
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Myeloproliferative Disorders / drug therapy*
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Myeloproliferative Disorders / genetics
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Platelet Count
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Pyrazines / therapeutic use*
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Thrombocytosis / complications
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Thrombocytosis / drug therapy*
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Thrombocytosis / genetics
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Tumor Necrosis Factor-alpha / metabolism
Substances
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Antineoplastic Agents
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Boronic Acids
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Interleukin-6
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Pyrazines
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Tumor Necrosis Factor-alpha
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Interleukin-4
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Bortezomib