Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report

Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma, marked by liver, spleen, and bone marrow sinusoidal infiltration, with an aggressive clinical course, which represents a difficult diagnostic task for clinicians and pathologists. Another equally severe and challenging condition i...

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Main Authors: Vitor Ribeiro Paes, Patrícia Picciarelli de Lima, Sheila Aparecida Coelho Siqueira
Format: Article
Language:English
Published: University of São Paulo 2014-12-01
Series:Autopsy and Case Reports
Subjects:
Online Access:http://www.revistas.usp.br/autopsy/article/view/98462
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author Vitor Ribeiro Paes
Patrícia Picciarelli de Lima
Sheila Aparecida Coelho Siqueira
author_facet Vitor Ribeiro Paes
Patrícia Picciarelli de Lima
Sheila Aparecida Coelho Siqueira
author_sort Vitor Ribeiro Paes
collection DOAJ
description Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma, marked by liver, spleen, and bone marrow sinusoidal infiltration, with an aggressive clinical course, which represents a difficult diagnostic task for clinicians and pathologists. Another equally severe and challenging condition is the hemophagocytic lymphohistiocytosis (also called hemophagocytic syndrome [HS]), which is often associated with hematologic malignancies and infectious diseases. The authors report the case of a 56-year-old woman diagnosed with HSTCL based on bone marrow aspirate flow cytometry and skin biopsy. The patient underwent a cycle of chemotherapy but the outcome was unfavorable with multiple organ failure. The laboratory analysis was consistent with HS. The autopsy confirmed both the remaining lymphoma in the pulmonary vessels and the hemophagocytic cells in the spleen and bone marrow.
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spelling doaj.art-0b92104bfef345d49d244413b00298922022-12-21T22:04:20ZengUniversity of São PauloAutopsy and Case Reports2236-19602014-12-014410.4322/acr.%y.9846288480Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case reportVitor Ribeiro Paes0Patrícia Picciarelli de Lima1Sheila Aparecida Coelho Siqueira2Pathological Anatomical Division - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SPPathological Anatomical Division - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SPPathological Anatomical Division - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SPHepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma, marked by liver, spleen, and bone marrow sinusoidal infiltration, with an aggressive clinical course, which represents a difficult diagnostic task for clinicians and pathologists. Another equally severe and challenging condition is the hemophagocytic lymphohistiocytosis (also called hemophagocytic syndrome [HS]), which is often associated with hematologic malignancies and infectious diseases. The authors report the case of a 56-year-old woman diagnosed with HSTCL based on bone marrow aspirate flow cytometry and skin biopsy. The patient underwent a cycle of chemotherapy but the outcome was unfavorable with multiple organ failure. The laboratory analysis was consistent with HS. The autopsy confirmed both the remaining lymphoma in the pulmonary vessels and the hemophagocytic cells in the spleen and bone marrow.http://www.revistas.usp.br/autopsy/article/view/98462LymphomaT-cellPeripheralLymphohistiocytosisHemophagocyticAutopsy
spellingShingle Vitor Ribeiro Paes
Patrícia Picciarelli de Lima
Sheila Aparecida Coelho Siqueira
Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
Autopsy and Case Reports
Lymphoma
T-cell
Peripheral
Lymphohistiocytosis
Hemophagocytic
Autopsy
title Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
title_full Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
title_fullStr Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
title_full_unstemmed Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
title_short Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report
title_sort hemophagocytic lymphohistiocytosis associated with hepatosplenic t cell lymphoma case report
topic Lymphoma
T-cell
Peripheral
Lymphohistiocytosis
Hemophagocytic
Autopsy
url http://www.revistas.usp.br/autopsy/article/view/98462
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AT patriciapicciarellidelima hemophagocyticlymphohistiocytosisassociatedwithhepatosplenictcelllymphomacasereport
AT sheilaaparecidacoelhosiqueira hemophagocyticlymphohistiocytosisassociatedwithhepatosplenictcelllymphomacasereport