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.