Aplastic anaemia with 13q–: a benign subset of bone marrow failure responsive to immunosuppressive therapy

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...

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Bibliographic Details
Published in:British journal of haematology 2002-06, Vol.117 (3), p.747-750
Main Authors: Ishiyama, Ken, Karasawa, Masamitsu, Miyawaki, Shuichi, Ueda, Yasunori, Noda, Masaaki, Wakita, Atsushi, Sawanobori, Masakazu, Nagai, Hirokazu, Nakao, Shinji
Format: Article
Language:English
Subjects:
13q deletion
Adult
Aged
Anemia, Aplastic - genetics
Anemia, Aplastic - therapy
aplastic anaemia
benign clinical course
Biological and medical sciences
Chromosome Deletion
Chromosomes, Human, Pair 13
Drug toxicity and drugs side effects treatment
Female
Follow-Up Studies
Hematology
Humans
Immunosuppressive Agents - therapeutic use
immunosup‐ pressive therapy
innocent clones
Male
Medical sciences
Middle Aged
Pharmacology. Drug treatments
Prognosis
Retrospective Studies
Survival Rate
Toxicity: blood
Treatment Outcome
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Summary: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.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2002.03518.x