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Title: RARS with fibrosis and del(20q) transformed into ALL
Authors: Rohr, Sandra S. [UNIFESP]
Flores Pelloso, Luis Arthur [UNIFESP]
Borgo, Aline dos Santos [UNIFESP]
Rezende, Joao Garibaldi de [UNIFESP]
Regis Silva, Maria Regina [UNIFESP]
Yamamoto, Mihoko [UNIFESP]
Chauffaille, Maria de Lourdes L. F. [UNIFESP]
Universidade Federal de São Paulo (UNIFESP)
Keywords: MDS with fibrosis
MDS transformation
Secondary ALL
Issue Date: 1-Dec-2012
Publisher: Humana Press Inc
Citation: Medical Oncology. Totowa: Humana Press Inc, v. 29, n. 5, p. 3570-3573, 2012.
Abstract: Transformation of myelodysplastic syndrome (MDS) into acute myelogenous leukemia occurs in approximately 30 % of cases, while progression into acute lymphoblastic leukemia (ALL) is rare. We report on a 67-year-old man with the diagnosis of MDS, subtype refractory anemia with ring sideroblasts (RARS), karyotype 20q- , JAK-2 negative and grade III fibrosis on the bone marrow biopsy, who evolved into ALL 33 months after the diagnosis of MDS. RARS is one of the subtypes of MDS with most indolent course. Deletion of the long arm of chromosome 20 (20q-) is considered as good prognosis by the International Prognostic Scoring System, an important scoring system for predicting survival and evolution of MDS. Primary MDS with bone marrow fibrosis may represent a distinct clinicopathological and is supposed to have an unfavorable prognosis. the combined analysis of these features makes this rare report still more challenging and illustrates that biology of MDS is yet to be discovered.
ISSN: 1357-0560
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