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Transformation of the 5q- syndrome to acute lymphoblastic leukemia: a report of two cases and review of the literature.
MedLine Citation:
PMID:  21487529     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Myelodysplastic syndrome (MDS) with an isolated deletion of the long arm of chromosome 5 (5q- syndrome) is a distinct subtype of MDS with an indolent course that rarely transforms to acute leukemia. Deletion of the long arm of chromosome 5 has also been reported in rare cases of de novo B-lymphoblastic leukemia. We present two cases of 5q- syndrome with a similar and unusual course of transformation to lymphoblastic leukemia while on Lenalidomide. These two patients achieved an initial response; however, later acquired a second cytogenetic abnormality, became refractory to treatment and evolved into acute leukemia. At the time of transformation, both patients had recurrence of the 5q- abnormality. Review of the literature and the mechanisms of transformation of the 5q-syndrome into an acute leukemia are discussed. Although the relationship between the events in our cases remains unclear, the intriguing similarity between the two cases raises a question whether immune modulators can alter the natural course of MDS. To our knowledge, no similar cases were previously reported in the literature.
Authors:
Nicole M Agostino; Basil Ahmed; Dan Popescu; Shereen Gheith
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2011-03-22
Journal Detail:
Title:  International journal of clinical and experimental pathology     Volume:  4     ISSN:  1936-2625     ISO Abbreviation:  Int J Clin Exp Pathol     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-04-13     Completed Date:  2011-08-12     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  101480565     Medline TA:  Int J Clin Exp Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  322-6     Citation Subset:  IM    
Affiliation:
Lehigh Valley Hospital and Health Network, Department of Hematology/Oncology, Allentown, PA 18105, USA. Nicole_M.Agostino@lvhn.org
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MeSH Terms
Descriptor/Qualifier:
Aged
Aged, 80 and over
Anemia, Macrocytic / genetics
Chromosome Aberrations
Chromosome Deletion
Chromosomes, Human, Pair 5 / genetics
Female
Humans
Male
Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
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