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Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman

Haemophagocytic lymphohistiocytosis is a syndrome of excessive immunological activation that can be triggered by various diseases, including haematological cancers. We report a case of a 25-year-old woman presenting with constitutional symptoms and a painful thoracic mass of four months duration. La...

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Autores principales: Nogueira, Fernando, Brito, Isabel C, Pereira, Catarina V, Marques, José C, Ferreira, Ester, Carneiro, Ana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10026187/
https://www.ncbi.nlm.nih.gov/pubmed/36945277
http://dx.doi.org/10.7759/cureus.35130
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author Nogueira, Fernando
Brito, Isabel C
Pereira, Catarina V
Marques, José C
Ferreira, Ester
Carneiro, Ana
author_facet Nogueira, Fernando
Brito, Isabel C
Pereira, Catarina V
Marques, José C
Ferreira, Ester
Carneiro, Ana
author_sort Nogueira, Fernando
collection PubMed
description Haemophagocytic lymphohistiocytosis is a syndrome of excessive immunological activation that can be triggered by various diseases, including haematological cancers. We report a case of a 25-year-old woman presenting with constitutional symptoms and a painful thoracic mass of four months duration. Laboratory exams showed pancytopenia, hypertriglyceridemia and extremely high serum ferritin levels. A whole-body computed tomography (CT) scan revealed splenomegaly and highlighted the mass on the deep tissues of the left breast; the biopsy was compatible with anaplastic large-cell lymphoma. Additionally, a bone marrow biopsy revealed haemophagocytosis, fulfilling the criteria for associated haemophagocytic lymphohistiocytosis. The patient was quickly sent for chemotherapy followed by autologous haematopoietic cell transplantation. She achieved a complete metabolic response and has been in clinical remission after nearly four years of follow-up. We emphasise the benefit of a timely diagnosis and intervention which were the keys to success in this case.
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spelling pubmed-100261872023-03-20 Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman Nogueira, Fernando Brito, Isabel C Pereira, Catarina V Marques, José C Ferreira, Ester Carneiro, Ana Cureus Internal Medicine Haemophagocytic lymphohistiocytosis is a syndrome of excessive immunological activation that can be triggered by various diseases, including haematological cancers. We report a case of a 25-year-old woman presenting with constitutional symptoms and a painful thoracic mass of four months duration. Laboratory exams showed pancytopenia, hypertriglyceridemia and extremely high serum ferritin levels. A whole-body computed tomography (CT) scan revealed splenomegaly and highlighted the mass on the deep tissues of the left breast; the biopsy was compatible with anaplastic large-cell lymphoma. Additionally, a bone marrow biopsy revealed haemophagocytosis, fulfilling the criteria for associated haemophagocytic lymphohistiocytosis. The patient was quickly sent for chemotherapy followed by autologous haematopoietic cell transplantation. She achieved a complete metabolic response and has been in clinical remission after nearly four years of follow-up. We emphasise the benefit of a timely diagnosis and intervention which were the keys to success in this case. Cureus 2023-02-18 /pmc/articles/PMC10026187/ /pubmed/36945277 http://dx.doi.org/10.7759/cureus.35130 Text en Copyright © 2023, Nogueira et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Nogueira, Fernando
Brito, Isabel C
Pereira, Catarina V
Marques, José C
Ferreira, Ester
Carneiro, Ana
Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title_full Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title_fullStr Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title_full_unstemmed Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title_short Haemophagocytic Lymphohistiocytosis Associated With Anaplastic Large-Cell Lymphoma in a Young Woman
title_sort haemophagocytic lymphohistiocytosis associated with anaplastic large-cell lymphoma in a young woman
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10026187/
https://www.ncbi.nlm.nih.gov/pubmed/36945277
http://dx.doi.org/10.7759/cureus.35130
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