Refractory anemia with ringed sideroblasts with a low IPSS score progressed rapidly with de novo appearance of multiple karyotypic abnormalities and into acute erythroleukemia (AML-M6A)

We report here a case of refractory anemia with ringed sideroblasts (RARS) with a low risk group by the International Prognostic Scoring System (IPSS) at the time of diagnosis but had a rapid disease progression. Although the patient showed a normal male karyotype at the time of RARS diagnosis, his...

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Bibliographic Details
Published in:Leukemia research 2000-07, Vol.24 (7), p.597-600
Main Authors: Iwase, Osamu, Iwama, Hirosi, Okabe, Seiichi, Ando, Keiko, Yaguchi, Makoto, Miyazawa, Keisuke, Kimura, Yukihiko, Kodama, Atsushi, Fukutake, Katsuyuki, Ohyashiki, Kazuma
Format: Article
Language:English
Subjects:
Acute Disease
Anemia, Refractory - genetics
Anemia, Refractory - physiopathology
Anemia, Sideroblastic - etiology
Anemia, Sideroblastic - genetics
Biological and medical sciences
Chromosome Aberrations
Chromosome Disorders
Disease Progression
Hematologic and hematopoietic diseases
Humans
Karyotypic abnormalities
Karyotyping
Leukemia, Erythroblastic, Acute - etiology
Leukemia, Erythroblastic, Acute - genetics
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Male
Medical sciences
Middle Aged
Myelodysplastic syndrome
Refractory anemia with ringed sideroblasts
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Summary:We report here a case of refractory anemia with ringed sideroblasts (RARS) with a low risk group by the International Prognostic Scoring System (IPSS) at the time of diagnosis but had a rapid disease progression. Although the patient showed a normal male karyotype at the time of RARS diagnosis, his marrow cells had del(5)(q14) and add(17)(p12) abnormalities 2 months after the diagnosis, and later the marrow cells had multiple abnormalities and the patient expired 6 months after the initial diagnosis of RARS. The patient was diagnosed as having RARS with a low risk group by the IPSS classification, however, one should keep in mind that some patients with myelodysplastic syndromes with low risks by either the French–American–British (FAB) classification or the IPSS classification may have progressive disease and subsequential cytogenetic analysis could predict the disease progression.
ISSN:0145-2126
1873-5835
DOI:10.1016/S0145-2126(00)00029-1