title: Aplastic anaemia with 13q-: a benign subset of bone marrow failure responsive to immunosuppressive therapy.
abstract: In an attempt to determine the pathological significance of a long arm deletion of chromosome 13 (13q-) in bone marrow failure syndrome, we reviewed the clinical records of nine patients who were initially diagnosed with aplastic anaemia due to bone marrow hypoplasia without dysplasia. Six patients responded to immunosuppressive therapy and the other three improved with steroids. None of the patients developed acute leukaemia (follow up: 54-129 months) and the estimated 5-year survival was 78%. These findings indicate that pancytopenia with 13q- represents bone marrow failure of a benign nature, similar to aplastic anaemia without karyotypic abnormalities, rather than preleukaemia.
authors.affiliation: Cellular Transplantation Biology, Division of Cancer Medicine, Kanazawa University Graduate School of Medical Science, 13-1 Takaramachi, Kanazawa, Ishikawa 920-8641, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Ishiyama
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Karasawa
authors.forename: Shuichi
authors.initials: S
authors.lastname: Miyawaki
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ueda
authors.forename: Masaaki
authors.initials: M
authors.lastname: Noda
authors.forename: Atsushi
authors.initials: A
authors.lastname: Wakita
authors.forename: Masakazu
authors.initials: M
authors.lastname: Sawanobori
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Nagai
authors.forename: Shinji
authors.initials: S
authors.lastname: Nakao
mesh_terms: D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000235:genetics* / Q000628:therapy; D002872:Chromosome Deletion; D002882:Chromosomes, Human, Pair 13; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents