Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, and systemic hyperinflammatory syndrome with exacerbated and uncontrolled activation of histiocytes and lymphocytes against mature cells. Secondary HLH can occur in association with a myriad of underlying infections or malignanci...

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Main Authors: Ahmed Arfa, Nivin Omar, Kulsum Bano, Natasha M. Savage
Format: Article
Language:English
Published: Wiley 2022-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2022/4669025
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author Ahmed Arfa
Nivin Omar
Kulsum Bano
Natasha M. Savage
author_facet Ahmed Arfa
Nivin Omar
Kulsum Bano
Natasha M. Savage
author_sort Ahmed Arfa
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, and systemic hyperinflammatory syndrome with exacerbated and uncontrolled activation of histiocytes and lymphocytes against mature cells. Secondary HLH can occur in association with a myriad of underlying infections or malignancies. Our patient is a 38-year-old male prisoner with poorly controlled diabetes and no known other medical conditions. He was referred to our emergency department with three-week history of worsening malaise, weight loss, fever, bruising, and shortness of breath; imaging showed pneumomediastinum, lung nodule, and adrenal mass. Biopsy of the lung nodule revealed acid-fast bacilli. Furthermore, bone marrow biopsy showed foci of necrosis with associated acid-fast bacilli and hemophagocytosis highlighted by CD163 stain; consequently, secondary HLH was suggested. Hence, lab results were reviewed and found to satisfy five of the eight secondary HLH criteria. Moreover, ferritin was >10,000 ng/ml, which has been suggested to be highly suspicious for HLH. The patient was started on anti-MAC therapy. Unfortunately, the patient’s status declined rapidly; he developed multi-organ failure and succumbed to disease. Later, his culture confirmed Mycobacterium tuberculosis. In conclusion, we presented a rare and challenging case of secondary HLH associated with disseminated Mycobacterium tuberculosis. A high index of suspicion is required for early diagnosis and treatment, and pathologists should be aware of Mycobacterium tuberculosis’ association with secondary HLH.
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spelling doaj-art-5b711fcb8f3b49418dd37ce23cdee32b2025-08-20T02:05:13ZengWileyCase Reports in Hematology2090-65792022-01-01202210.1155/2022/4669025Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic LymphohistiocytosisAhmed Arfa0Nivin Omar1Kulsum Bano2Natasha M. Savage3Department of PathologyDepartment of PathologyDepartment of PathologyDepartment of PathologyHemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, and systemic hyperinflammatory syndrome with exacerbated and uncontrolled activation of histiocytes and lymphocytes against mature cells. Secondary HLH can occur in association with a myriad of underlying infections or malignancies. Our patient is a 38-year-old male prisoner with poorly controlled diabetes and no known other medical conditions. He was referred to our emergency department with three-week history of worsening malaise, weight loss, fever, bruising, and shortness of breath; imaging showed pneumomediastinum, lung nodule, and adrenal mass. Biopsy of the lung nodule revealed acid-fast bacilli. Furthermore, bone marrow biopsy showed foci of necrosis with associated acid-fast bacilli and hemophagocytosis highlighted by CD163 stain; consequently, secondary HLH was suggested. Hence, lab results were reviewed and found to satisfy five of the eight secondary HLH criteria. Moreover, ferritin was >10,000 ng/ml, which has been suggested to be highly suspicious for HLH. The patient was started on anti-MAC therapy. Unfortunately, the patient’s status declined rapidly; he developed multi-organ failure and succumbed to disease. Later, his culture confirmed Mycobacterium tuberculosis. In conclusion, we presented a rare and challenging case of secondary HLH associated with disseminated Mycobacterium tuberculosis. A high index of suspicion is required for early diagnosis and treatment, and pathologists should be aware of Mycobacterium tuberculosis’ association with secondary HLH.http://dx.doi.org/10.1155/2022/4669025
spellingShingle Ahmed Arfa
Nivin Omar
Kulsum Bano
Natasha M. Savage
Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
Case Reports in Hematology
title Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
title_full Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
title_fullStr Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
title_full_unstemmed Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
title_short Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
title_sort disseminated mycobacterium tuberculosis an unusual presentation with associated hemophagocytic lymphohistiocytosis
url http://dx.doi.org/10.1155/2022/4669025
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AT kulsumbano disseminatedmycobacteriumtuberculosisanunusualpresentationwithassociatedhemophagocyticlymphohistiocytosis
AT natashamsavage disseminatedmycobacteriumtuberculosisanunusualpresentationwithassociatedhemophagocyticlymphohistiocytosis