Acute Promyelocytic Leukemia : Experience at a North Indian Tertiary Care Hospital with Review of Literature
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Date
2001-01
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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.
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Keywords
Ali-trans relinoic acid, Retinoic acid syndrome, Disseminated intravascular coagulation, RARa