Case Report Transformation of the 5q- syndrome to acute lymphoblastic leukemia: a report of two cases and review of the literature
Nicole M. Agostino, Basil Ahmed, Dan Popescu, Shereen Gheith
Lehigh Valley Hospital and Health Network, Department of Hematology/Oncology; Lehigh Valley Hospital and Health Network, Department of Pathology, Allentown, PA 18105, USA.
Received January 15, 2011; accepted March 9, 2011; Epub March 22, 2011; published March 31, 2011
Abstract: 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 been also 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 on 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. (IJCEP1101002).
Address all correspondence to: Nicole M Agostino, DO Lehigh Valley Hospital and Health Network 1240 S Cedar Crest Blvd. Suite 405 Allentown, PA 18105 Tel: 610-402-9104 E-mail: Nicole_M.Agostino@lvhn.org