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Extramedullary disease in acute promyelocytic leukaemia: A rare presentation

Acute promyelocytic leukaemia consists of 7%–8% of cases of acute myeloid leukaemia. Extramedullary manifestations are rare and show distinct biological features. We describe a 22-year-old female of Malay ethnicity who presented with fever and a left axillary swelling for a week. The peripheral bloo...

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Autores principales: Kasinathan, Ganesh, Sathar, Jameela
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7268156/
https://www.ncbi.nlm.nih.gov/pubmed/32537161
http://dx.doi.org/10.1177/2050313X20926076
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author Kasinathan, Ganesh
Sathar, Jameela
author_facet Kasinathan, Ganesh
Sathar, Jameela
author_sort Kasinathan, Ganesh
collection PubMed
description Acute promyelocytic leukaemia consists of 7%–8% of cases of acute myeloid leukaemia. Extramedullary manifestations are rare and show distinct biological features. We describe a 22-year-old female of Malay ethnicity who presented with fever and a left axillary swelling for a week. The peripheral blood smear showed abnormal promyelocytes with faggot cells. PML-RAR-alpha t(15;17) (q22; q12) was detected by polymerase chain reaction. The left axillary swelling histology and immunohistochemical staining confirmed granulocytic sarcoma. She was induced with triple agents consisting of all-trans-retinoic-acid, arsenic trioxide and idarubicin. On day 14 of induction, she developed severe neutropenic sepsis in which she responded to ventilation and antimicrobials. She completed her induction, consolidation and maintenance therapy. Currently she is in molecular and morphological remission. Extramedullary disease in acute promyelocytic leukaemia usually has a severe clinical presentation. Granulocytic sarcoma may present as an early feature of acute promyelocytic leukaemia.
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spelling pubmed-72681562020-06-11 Extramedullary disease in acute promyelocytic leukaemia: A rare presentation Kasinathan, Ganesh Sathar, Jameela SAGE Open Med Case Rep Case Report Acute promyelocytic leukaemia consists of 7%–8% of cases of acute myeloid leukaemia. Extramedullary manifestations are rare and show distinct biological features. We describe a 22-year-old female of Malay ethnicity who presented with fever and a left axillary swelling for a week. The peripheral blood smear showed abnormal promyelocytes with faggot cells. PML-RAR-alpha t(15;17) (q22; q12) was detected by polymerase chain reaction. The left axillary swelling histology and immunohistochemical staining confirmed granulocytic sarcoma. She was induced with triple agents consisting of all-trans-retinoic-acid, arsenic trioxide and idarubicin. On day 14 of induction, she developed severe neutropenic sepsis in which she responded to ventilation and antimicrobials. She completed her induction, consolidation and maintenance therapy. Currently she is in molecular and morphological remission. Extramedullary disease in acute promyelocytic leukaemia usually has a severe clinical presentation. Granulocytic sarcoma may present as an early feature of acute promyelocytic leukaemia. SAGE Publications 2020-06-02 /pmc/articles/PMC7268156/ /pubmed/32537161 http://dx.doi.org/10.1177/2050313X20926076 Text en © The Author(s) 2020 https://creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Kasinathan, Ganesh
Sathar, Jameela
Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title_full Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title_fullStr Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title_full_unstemmed Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title_short Extramedullary disease in acute promyelocytic leukaemia: A rare presentation
title_sort extramedullary disease in acute promyelocytic leukaemia: a rare presentation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7268156/
https://www.ncbi.nlm.nih.gov/pubmed/32537161
http://dx.doi.org/10.1177/2050313X20926076
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