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

Idarubicin Plus Behenoyl Cytarabine and 6-thioguanine Compares Favorably with Idarubicin Plus Cytarabine-based Regimen for Children with Previously Untreated Acute Myeloid Leukemia: 10-Year Retrospective, Multicenter Study in Korea

We investigated the outcome of idarubicin plus N4-behenoyl-1-beta-D-arabinofuranosyl cytosine (BHAC)-based chemotherapy (BHAC group, n=149) compared to idarubicin plus cytarabine-based chemotherapy (cytarabine group, n=191) for childhood acute myeloid leukemia (AML). Between January 1996 and December 2005, 340 children with AML from 5 university hospitals in Korea received the BHAC-based or cytarabine-based chemotherapy, with or without hematopoietic stem cell transplantation. After induction therapy, 264 (77.6%) of 340 children achieved a complete remission (CR) and 43 (12%) achieved a partial remission (PR). The CR rate in the BHAC group was higher than in the cytarabine group (85.2% vs. 71.7%, P=0.004). However, the overall response rate (CR+PR) was not different between the two groups (93.3% vs. 87.9%, P=0.139). The 5-yr estimates of overall survival (OS) of children in the two groups were similar (54.9% for the BHAC group vs. 52.4% for the cytarabine group, P=0.281). Although the results were analyzed according to the treatment type and cytogenetic risk, the OS showed no significant difference between the BHAC group and the cytarabine group. In the present study, the clinical outcomes of the BHAC-based chemotherapy, consisting of BHAC, idarubicin, and 6-TG, are comparable to that of the cytarabine-based chemotherapy for childhood AML.

Behenoyl cytarabine-associated reversible encephalopathy in a patient with acute myelogenous leukemia

We report a case of reversible encephalopathy syndrome in a 16-year-old girl with acute myelogenous leukemia (AML), who is undergoing during consolidation chemotherapy composed of BH-AC (N4-behenoyl-1-beta-D-arabinofuranosyl cytosine) and idarubicin. On the 6th day of chemotherapy, she was in a drowsy state following generalized tonic clonic seizure lasting 20 minutes. MR images revealed extensive cortical and subcortical white matter brain edema. Alertness returned over the 24 hr following by the discontinuation of BH-AC and intravenous administration of diphenylhydantoin, although she complained of intermittent headaches and visual disturbance. She gradually recovered from these symptoms during subsequent 7 days. Previously noted abnormal signal intensities have nearly disappreared on follow-up MRI obtained on the 22nd day after the first seizure. She was discharged without any neurologic sequela. This case suggests that BH-AC, a derivative of cytosine arabinoside (1-beta-D-arabinofuranosylcytosine) could be a cause of reversible encephalopathy syndrome.