Acute Promyelocytic Leukaemia: A Large Case Series from Southern India Based on Clinical, Pathological, Immunophenotypic, Cytogenetic and Molecular Studies

Autor: T SRAVANI, PAMU PRAMOD KUMAR, ASWANI TANDON, MEGHA S UPPIN, SHANTIVEER G UPPIN, AMVR NARENDRA, GUNDETI SADASHIVUDU, T ROSHNI PAUL
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Zdroj: National Journal of Laboratory Medicine, Vol 8, Iss 4, Pp PO08-PO12 (2019)
Druh dokumentu: article
ISSN: 2277-8551
2455-6882
DOI: 10.7860/NJLM/2019/42483:2365
Popis: Introduction: Acute Promyelocytic Leukaemia (APL/APML) is an uncommon haematologic malignancy associated with translocation t (15; 17) and presence of abnormal promyelocytes in the peripheral blood and bone marrow. Aim: To know the clinico-pathological profile of all cases diagnosed as APL and correlate the morphology with the cytogenetic and molecular studies. Materials and Methods: It was a retrospective cum prospective analytical study done over a period of five years. Initially all cases were diagnosed on basis of morphology and later confirmed by immuno-phenotyping and molecular characterisation. Results were analysed based on morphological distribution of the cases, Immuno-chemistry and cyto-genetics. Results: During the study period, 125 cases were diagnosed as APL. The patient’s ages ranged from 3 years-60 years (median age 35 years) with M: F ratio of 1.4:1. Common clinical presentations were fever, bleeding manifestations and anaemia. Total leucocytes count varied with cases; 56 cases presented with leucopenia, 44 cases with leucocytosis and 25 cases with normal leucocytes counts. Most of the cases showed marked thrombocytopenia. The cases were classified as hypergranular (80 cases), microgranular (39 cases) and APL Not Otherwise Specified (NOS) with atypical features (6 cases). Sudan Black B (SBB) was intensely positive in 100% of cases. Promyelocytic Leukaemia/Retinoic Acid Receptor Alpha (PML-RARA) by Flourescence In-Situ Hybridization (FISH) and Polymerase Chain Reaction (PCR) testing was positive in 95.5% of available cases. Other cases were diagnosed based on morphology, cytochemistry and response to All-trans Retinoic Acid/Arsenic Trioxide (ATRA/ ATO). Conclusion: APL stands apart from other AMLs because of its clinical features, morphology, and distinct cytogenetic and good response to therapy. Early diagnosis of APL is essential for management of patients for better outcome.
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