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Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis.

MedStar author(s):

Bhansali, Deepty

, MUMH

, Internal Medicine Residency

Nanjudappa, Athmananda

Citation: BMJ Case Reports. 15(8), 2022 Aug 24.PMID: 36002177Institution: MedStar Franklin Square Medical Center | MedStar Union Memorial HospitalDepartment: Hospitalist | Internal Medicine Residency | PathologyForm of publication: Journal ArticleMedline article type(s): Case Reports | Journal ArticleSubject headings: *Arthritis, Juvenile | *Gastroenteritis | *Lupus Erythematosus, Systemic | *Lymphohistiocytosis, Hemophagocytic | *Macrophage Activation Syndrome | Arthritis, Juvenile/co [Complications] | Female | Gastroenteritis/co [Complications] | Gastroenteritis/di [Diagnosis] | Humans | Lupus Erythematosus, Systemic/co [Complications] | Lymphohistiocytosis, Hemophagocytic/co [Complications] | Lymphohistiocytosis, Hemophagocytic/di [Diagnosis] | Macrophage Activation Syndrome/co [Complications] | Macrophage Activation Syndrome/et [Etiology] | Middle AgedYear: 2022 ISSN:

1757-790X

Name of journal: BMJ case reportsAbstract: Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus. Copyright © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.All authors: Bhansali D, Haas CJ, Iding J, Kolachana S, Malik A, Nanjudappa AFiscal year: FY2023 Digital Object Identifier:

https://dx.doi.org/10.1136/bcr-2022-250809

ORCID:

http://orcid.org/0000-0002-8940-3548

Date added to catalog: 2022-10-20

Holdings ( 1 )
Title notes ( 2 )

Holdings
Item type	Current library	Collection	Call number	Status	Date due	Barcode
Journal Article	MedStar Authors Catalog	Article	36002177	Available		36002177

Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus. Copyright © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.

English