Blood Malignancies and Treatment

Biography

Biography: Aleem Jan

Abstract

The success story of childhood acute lymphoblastic leukemia, has transformed the outlook of Haem –oncology and correction of coagulopathy as a medical emergency targetting fibrinogen levels in safe range with concommitant start of ATRA + anthracyclines or Arsenic trioxide is replicating this success story in APML. Acute promyelocytic leukemia( APML) is a unique subset of acute myeloid leukemia with first description as unique entity in 1957.The disease is identified by distinctive morphology and a balanced reciprocal translocation between chromosome(t15:17). Historically Acute promyelocytic leukemia is charecterised by a rapidly fatal course with high incidence of early death. Current recommendations are that when a diagnosis of APL is suspected based upon clinical presentation and or morphology, the disease should be treated as medical emergency. Urgent administration of ATRA should be instituted with aggressive supportive care including blood product support with platelets and cryoprecitate while genetic diagnosis is established. Risk stratification is imperative in the treatment of APL patients as those with low risk disease (WBC <10,000, platelet> 40,000) are generally treated with less intensive protocol than those presenting with high risk disease ( W.B.C >10,000) corresponding to initial criteria by.Sanz et al of WBC 10,000 and platelet 40,000. We at our centre do also look at CD 56 and BCR2. APL has evolved from ATRA+ chemotherapy for all patients to addition of Aresenic trioxide (ATO) to ATRA with omission of chemotherapy.. We at our centre are treating APML with ICP 2006 protocol which has ATRA + Anthracycline in induction with Dexamethasone for 2 weeks if counts more than 5,000. Dose of ATRA for paediatric population is 25mg/m2. Following induction we employ three consolidation blocks and in high risk cases cytosine arabinoside is incorporated. Molecular remission is documented by RQ PCR for PMLRAR α before maintenance for 2 years with 6 mercaptopurine weekly methotrexate and 3 monthly ATRA for 15 days.We believe in resource depleted countries and in camatose patients ATO is no inferior drug.it is administered in dose of 0.15mg/kg body weight until remission with close monitoring of APL syndrome and complications by weekly LFT,KFT,coagulogram,ECG for QT intervel. After documenting molecular remission maintenance is given for six months with same dose daily for 10 days. Over a period of 5 years we treated 57 cases of APML out of which 12 were paediatric cases ATRA + chemo as well as ATO based protocols were used.Results are encouraging and worth sharing.in meet. We do have relapse /refractory cases in whom ATRA +ATO is employed till remission. Given logistic difficulties in procuring anti CD 33(GO), autologous in PCR negative and allogenic BMT in PCR positive cases is undertaken.