Ph+ MDS – pro
Myelodysplastic syndromes (MDSs) are characteristically defined by the presence of specific karyotypic abnormalities, based on which they have been prognosticated. Translocation t(9;22)(q34;q11.2) (Philadelphia positive [Ph +ve]) and corresponding BCR-ABL fusion transcript is the defining parameter of chronic myeloid leukemia. It is also seen in a fair proportion of adult acute lymphoblastic leukemia. Occurrence of a Ph +ve MDS is very uncommon, and that too is seen mostly on progression to higher stage/acute leukemia. Even rarer is the de novo presence of Ph positivity in an MDS. A literature search through PubMed has shown only about forty cases of Ph +ve MDS among which less than half had shown Ph positivity at the time of initial diagnosis. As might be expected, this condition is the stuff of case reports and there is no informaiton on how to treat it, whether it requires treatment at all, or whether PH+ directed therpay is of any value.
Ling Zhang, Uncommon of the Uncommon: Low-Grade Myelodysplastic Syndrome Evolving Into Chronic Myelogenous Leukemia. Journal of Clinical Oncology 2011 29:15, e434-e436
Khaliqur Rahman et al, De novo Philadelphia chromosome positive myelodysplastic syndrome: Report of two cases with brief literature review
http://www.cancerjournal.net/preprintarticle.asp?id=188428;type=0