title: Bortezomib is an effective agent for MDS/MPD syndrome with 5q- anomaly and thrombocytosis.
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.
authors.affiliation: Department of Hematology and Medical Research, 251 General Airforce Hospital, 3 Kanellopoulou Street, GR-11525, Athens, Greece. eterpos@hotmail.com
authors.forename: Evangelos
authors.initials: E
authors.lastname: Terpos
authors.forename: Evgenia
authors.initials: E
authors.lastname: Verrou
authors.forename: Anastasia
authors.initials: A
authors.lastname: Banti
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Kaloutsi
authors.forename: Anna
authors.initials: A
authors.lastname: Lazaridou
authors.forename: Kostas
authors.initials: K
authors.lastname: Zervas
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001897:Boronic Acids / Q000627:therapeutic use*; D000069286:Bortezomib; D002895:Chromosomes, Human, Pair 5 / Q000235:genetics*; D005260:Female; D006801:Humans; D015847:Interleukin-4 / Q000378:metabolism; D015850:Interleukin-6 / Q000378:metabolism; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D009196:Myeloproliferative Disorders / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D010976:Platelet Count; D011719:Pyrazines / Q000627:therapeutic use*; D013922:Thrombocytosis / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D014409:Tumor Necrosis Factor-alpha / Q000378:metabolism
chemical_list: D000970:Antineoplastic Agents; D001897:Boronic Acids; D015850:Interleukin-6; D011719:Pyrazines; D014409:Tumor Necrosis Factor-alpha; D015847:Interleukin-4; D000069286:Bortezomib