Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma

Hemophagocytic lymphohistiocytosis (HLH) is a result of an abnormal activation of immune cells (T lymphocytes, natural killer cells, and macrophages) resulting in cytokine overproduction and immune destruction of cells, eventually resulting in multiorgan failure. Genetic causes are responsible for p...

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Main Authors: Oluwadunni E. Emiloju, Sorab Gupta, Vivian Arguello-Guerra, Claudia Dourado
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2019/7634760
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author Oluwadunni E. Emiloju
Sorab Gupta
Vivian Arguello-Guerra
Claudia Dourado
author_facet Oluwadunni E. Emiloju
Sorab Gupta
Vivian Arguello-Guerra
Claudia Dourado
author_sort Oluwadunni E. Emiloju
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a result of an abnormal activation of immune cells (T lymphocytes, natural killer cells, and macrophages) resulting in cytokine overproduction and immune destruction of cells, eventually resulting in multiorgan failure. Genetic causes are responsible for primary hemophagocytosis, but malignancies, infections, and autoimmunity underlie most of the secondary cases. We present an unusual case of a patient with AIDS and disseminated Kaposi sarcoma who was commenced on highly active antiretroviral therapy (HAART) but developed HLH secondary to immune reconstitution inflammatory syndrome (IRIS). We report this case to highlight the difficulty in managing this patient given the complex interplay of immunosuppression due to AIDS, immune reconstitution following initiation of HAART, and immune overdrive manifesting as HLH.
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id doaj-art-43ec2505bc784532abc434f893b650c2
institution Kabale University
issn 2090-6560
2090-6579
language English
publishDate 2019-01-01
publisher Wiley
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series Case Reports in Hematology
spelling doaj-art-43ec2505bc784532abc434f893b650c22025-02-03T06:07:12ZengWileyCase Reports in Hematology2090-65602090-65792019-01-01201910.1155/2019/76347607634760Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment DilemmaOluwadunni E. Emiloju0Sorab Gupta1Vivian Arguello-Guerra2Claudia Dourado3Department of Medicine, Einstein Medical Center, Philadelphia, PA 19141, USADepartment of Hematology and Medical Oncology, Einstein Medical Center, Philadelphia, PA 19141, USADepartment of Pathology and Laboratory Medicine, Einstein Medical Center, Philadelphia, PA 19141, USADepartment of Hematology and Medical Oncology, Einstein Medical Center, Philadelphia, PA 19141, USAHemophagocytic lymphohistiocytosis (HLH) is a result of an abnormal activation of immune cells (T lymphocytes, natural killer cells, and macrophages) resulting in cytokine overproduction and immune destruction of cells, eventually resulting in multiorgan failure. Genetic causes are responsible for primary hemophagocytosis, but malignancies, infections, and autoimmunity underlie most of the secondary cases. We present an unusual case of a patient with AIDS and disseminated Kaposi sarcoma who was commenced on highly active antiretroviral therapy (HAART) but developed HLH secondary to immune reconstitution inflammatory syndrome (IRIS). We report this case to highlight the difficulty in managing this patient given the complex interplay of immunosuppression due to AIDS, immune reconstitution following initiation of HAART, and immune overdrive manifesting as HLH.http://dx.doi.org/10.1155/2019/7634760
spellingShingle Oluwadunni E. Emiloju
Sorab Gupta
Vivian Arguello-Guerra
Claudia Dourado
Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
Case Reports in Hematology
title Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
title_full Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
title_fullStr Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
title_full_unstemmed Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
title_short Hemophagocytic Lymphohistiocytosis in an AIDS Patient with Kaposi Sarcoma: A Treatment Dilemma
title_sort hemophagocytic lymphohistiocytosis in an aids patient with kaposi sarcoma a treatment dilemma
url http://dx.doi.org/10.1155/2019/7634760
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AT vivianarguelloguerra hemophagocyticlymphohistiocytosisinanaidspatientwithkaposisarcomaatreatmentdilemma
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