Acute Promyelocytic Leukemia : Experience at a North Indian Tertiary Care Hospital with Review of Literature

ABSTRACT

Acute promyelocytic leukemia (APL) is characterised by balanced translocation between the long arms of chromoso)ne 15 and 17 resulting in formation of fusion protein PML/RARa. Due to this abnormal fusion protein, myeloid cell differentiation is arrested at the promyelocyte level. This molecular defect and myeloid cell differentiation arrest can be overcome by pharmacologic doses of ali-trans retinoic acid (ATRA). APL most common Iy presents as catastrophic bleeding manifestations which is a major cause of mortality. If diagnosed and treated early, patients can be salvaged and can achieve long term disease free survival. Our experience of seven patients is presented. All patiens presented with bleeding manifestation and two died due to it. Rest of the five patients who underwent chemotherapy in the form of induction with ATRA along with supportive measures (fresh frozen plasma and platelets) followed by consolidation therapy in the form of multi-agent chemotherapy, achieved prolonged disease free remission. Thus with early diagnosis and start of ATRA, APL is a potentially curable malignancy.