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Acute Promyelocytic Leukemia

Keywords: Ali-trans relinoic acid , Retinoic acid syndrome , Disseminated intravascular coagulation , RARa.Acute promyelocytic leukemia (APL) is characterised by balanced translocation between the long

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Abstract:

Acute promyelocytic leukemia (APL) is characterised by balanced translocation between the longarms of chromoso)ne 15 and 17 resulting in formation of fusion protein PMLlRARa. Due to thisabnormal fusion protein, myeloid cell differentiation is arrested at the promyelocyte level. Thismolecular defect and myeloid cell differentiation arrest can be overcome by pharmacologic doses ofali-trans retinoic acid (ATRA). APl most common Iy presents as catastroph ic bleed ing man ifestat ionswhich is a major cause of mortality. Ifdiagnosed and treated early, patients can be salvaged and canachieve long term disease free survival. Our experience of seven patients is presented. All patieillspresented with bleeding manifestation and two died due to it. Rest ofthe five patients who underwentchemotherapy in the form of induction with ATRA along with supportive measures (fresh frozenplasma 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 apotentially curable malignancy.

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