TY - JOUR AU - Jameel, Abid PY - 2011/12/29 Y2 - 2024/03/29 TI - PHILADELPHIA CHROMOSOME POSITIVE ACUTE MYELOID LEUKEMIA: A RARE HEMATOLOGICAL DISORDER: A CASE REPORT JF - Journal of Postgraduate Medical Institute JA - J Postgrad Med Inst VL - 26 IS - 1 SE - Case Report DO - UR - https://www.jpmi.org.pk/index.php/jpmi/article/view/1194 SP - AB - <em><span style="font-family: TimesNewRomanPSMT,Italic; font-size: x-small;"><em><span style="font-family: TimesNewRomanPSMT,Italic; font-size: x-small;"><p>Philadelphia chromosome positive (Ph-positive) acute myeloid leukemia (AML) is an extremely rare and</p><p>aggressive disease constituting approximately 1-3% of all de-novo AML cases. This disorder has many</p><p>features distinct from chronic myeloid leukemia (CML) in blast crisis (CML-BC) and is therefore</p><p>considered a distinct entity. Patients with Ph-positive AML have lower peripheral basophilia, lower bone</p><p>marrow cellularity and lower myeloid/erythroid ratio. Presentation is acute with a short history and these</p><p>patients are less likely to have splenomegaly. Outcome of the disease is poor and median overall survival</p><p>is 6-9 months. This disease shows resistance to conventional chemotherapy protocols. We have identified</p><p>two cases of Ph-positive AML amongst all de-novo AML patients diagnosed in our unit from January 2006</p><p>to December 2010. Both were treated with two courses of Cytarabine and Daunorubicin followed by</p><p>Imatinib Mesylate (IM) 600 mg orally daily. Patient no. 1 did not respond to two cycles of chemotherapy</p><p>as well as IM 600 mg daily and died after 5 months. Patient no. 2 had a complete hematologic response</p><p>after two cycles of chemotherapy along with IM and remained in full hematological remission with IM 600</p><p>mg daily maintenance for 7 months after diagnosis. After 7 months he had a relapse and died after 2</p><p>months of relapse. Combination of AML type of chemotherapy and maintenance with IM provides short</p><p>term remission while allogeneic stem cell transplant (ASCT) may achieve long term survival in a few</p><p>patients.</p></span></em></span></em> ER -