Bortezomib is an effective agent for MDS/MPD syndrome with 5q− anomaly and thrombocytosis

• Published in: Leukemia research 2007-04, Vol.31 (4), p.559-562
• Main Authors: Terpos, Evangelos, Verrou, Evgenia, Banti, Anastasia, Kaloutsi, Vassiliki, Lazaridou, Anna, Zervas, Kostas
• Format: Article
• Language: English
• Subjects: 5q− syndrome; Antineoplastic Agents - therapeutic use; Boronic Acids - therapeutic use; Bortezomib; Chromosomes, Human, Pair 5 - genetics; Female; Hematology, Oncology and Palliative Medicine; Humans; Interleukin-4; Interleukin-4 - metabolism; Interleukin-6; Interleukin-6 - metabolism; Middle Aged; Myelodysplastic syndromes; Myelodysplastic Syndromes - complications; Myelodysplastic Syndromes - drug therapy; Myelodysplastic Syndromes - genetics; Myeloproliferative Disorders - complications; Myeloproliferative Disorders - drug therapy; Myeloproliferative Disorders - genetics; Platelet Count; Pyrazines - therapeutic use; Thrombocytosis; Thrombocytosis - complications; Thrombocytosis - drug therapy; Thrombocytosis - genetics; Tumor Necrosis Factor-alpha - metabolism
• ISSN: 0145-2126; 1873-5835
• DOI: 10.1016/j.leukres.2006.05.018

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-α 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.