Philadelphia chromosome positive acute myeloid leukemia: a rare hematological disorder: a case report

ABSTRACT

Philadelphia chromosome positive [Ph-positive] acute myeloid leukemia [AML] is an extremely rare and aggressive disease constituting approximately 1-3% of all de-novo AML cases. This disorder has many features distinct from chronic myeloid leukemia [CML] in blast crisis [CML-BC] and is therefore considered a distinct entity. Patients with Ph-positive AML have lower peripheral basophilia, lower bone marrow cellularity and lower myeloid/erythroid ratio. Presentation is acute with a short history and these patients are less likely to have splenomegaly. Outcome of the disease is poor and median overall survival is 6-9 months. This disease shows resistance to conventional chemotherapy protocols. We have identified two cases of Ph-positive AML amongst all de-novo AML patients diagnosed in our unit from January 2006 to December 2010. Both were treated with two courses of Cytarabine and Daunorubicin followed by Imatinib Mesylate [IM] 600 mg orally daily. Patient no.1 did not respond to two cycles of chemotherapy as well as IM 600 mg daily and died after 5 months. Patient no.2 had a complete hematologic response after two cycles of chemotherapy along with IM and remained in full hematological remission with IM 600 mg daily maintenance for 7 months after diagnosis. After 7 months he had a relapse and died after 2 months of relapse. Combination of AML type of chemotherapy and maintenance with IM provides short term remission while allogeneic stem cell transplant [ASCT] may achieve long term survival in a few patients