A case series of patients with de novo Philadelphia-positive acute myeloid leukemia at a tertiary care center in South India - clinical profile, outcomes, and review of literature

Abstract

Introduction: Ph+ AML is a well-recognized , but rare entity that poses challenges to clinicians to differentiate it from Chronic myeloid leukemia in blast crisis. Objective: Our aim was to study the clinical profile, treatment and outcomes of patients with Ph+ AML at our centre. Materials and Methods: Four cases of Ph+ AML entered in the hospital registry between 2013 and 2015 were identified and analysed. Results: The median age was 46 years with equal sex preponderance. The duration of presenting complaints was <3 months. Median presenting total count was 54,000 cells/cumm. 3 patients had mild splenomegaly. None had basophilia. There was no predominant FAB-subtype. The cytogenetics revealed abnormalities in addition to the Philadelphia chromosome in 2 patients.3 out of 4 patients received induction chemotherapy with 3+7 - cytosine arabinoside and daunomycin alon with Imatinib dosed at 400 mg/ day. They were all in hematological, morphological and cytogenetic remission following induction. None underwent allogenic stem cell transplant. Of the 4, one refused treatment and is lost to follow up. Of those who received induction chemotherapy, 2 received consolidation with high dose ARAC, of which- one completed consolidation (4 courses) and is in remission 27 months after diagnosis, and the other has completed 1st high dose consolidation (and is alive 3 months after diagnosis) and the third one received 3 courses of low dose subcutaneous ARAC due to poor tolerability and expired 7 months after diagnosis. Conclusions: Ph+ AML is rare with no clear treatment recommendations. Improved outcomes with tyrosine kinase inhibitors(TKI's) and stem cell transplant should prompt further studies. If patients remain in remission with long term TKI use, it could serve as an alternative to stem cell transplantation in low income countries.