PHILADELPHIA CHROMOSOME POSITIVE ACUTE MYELOID LEUKEMIA: A RARE HEMATOLOGICAL DISORDER: A CASE REPORT

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