Hemophagocytic lymphohistiocytosis following enteric fever: A rare autopsy case report

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dc.contributor.authorFernandes, Gwendolynen_US
dc.contributor.authorMhashete, Poojaen_US
dc.contributor.authorPatwardhan, Pranav Pramoden_US
dc.date.accessioned2025-05-09T09:57:04Z
dc.date.available2025-05-09T09:57:04Z
dc.date.issued2024-06
dc.description.abstractHemophagocytic lymphohistiocytosis (HLH) is a severe and frequently underdiagnosed disorder of systemic immune dysregulation resulting in hypercytokinemia and histologically evident hemophagocytosis, We report a case of a 34?year?old man who presented with breathlessness, generalized weakness, and fever of unknown origin with pancytopenia. Clinically the patient was admitted for febrile illness, and treated symptomatically but his general condition worsened leading to death within 21 hours of admission. A complete autopsy was performed. The deceased had a significant past history of repeated episodes of fever, weight loss, and axillary lymphadenopathy over a period of 8 months with multiple hospital admissions. He was also diagnosed with enteric fever (Widal test and Typhi IgM positive) at the start of these episodes. Hemogram during this period revealed persistent pancytopenia. Serum ferritin, serum triglycerides, and liver function tests were consistently deranged. Investigations for the etiology of fever and blood cultures were negative while the bone marrow aspirate revealed a normocellular marrow. CT abdomen?pelvis showed mild hepatomegaly with enlarged retroperitoneal lymph nodes. Infective endocarditis, lymphoma, and bronchopneumonia were being considered the clinical diagnoses. The significant autopsy findings were hepatosplenomegaly with retroperitoneal lymphadenopathy and multiple gastric ulcers. On microscopy, the liver, spleen, bone marrow, and lymph nodes showed characteristic hemophagocytosis. Post?mortem histopathological examination clinched the diagnosis of HLH and fulfilled six out of eight diagnostic criteria of the HLH?2004 protocol. We discuss the clinical course and diagnosis of this unique case and strive to create awareness about secondary HLH induced by common diseases, such as enteric fever.en_US
dc.identifier.affiliationsDepartment of Pathology, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, Maharashtra, Indiaen_US
dc.identifier.affiliationsDepartment of Pathology, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, Maharashtra, Indiaen_US
dc.identifier.affiliationsDepartment of Pathology, Seth G.S. Medical College & K.E.M. Hospital, Mumbai, Maharashtra, Indiaen_US
dc.identifier.citationFernandes Gwendolyn, Mhashete Pooja, Patwardhan Pranav Pramod . Hemophagocytic lymphohistiocytosis following enteric fever: A rare autopsy case report. Indian Journal of Pathology & Microbiology. 2024 Jun; 67(2): 435-437en_US
dc.identifier.issn0377-4929
dc.identifier.issn0974-5130
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/245461
dc.languageenen_US
dc.publisherWolters Kluwer – Medknowen_US
dc.relation.issuenumber2en_US
dc.relation.volume67en_US
dc.source.urihttps://doi.org/10.4103/ijpm.ijpm_162_22en_US
dc.subjectHemophagocytosisen_US
dc.subjecthepatosplenomegalyen_US
dc.subjectmacrophage activation syndromeen_US
dc.subjectpyrexia of unknown originen_US
dc.titleHemophagocytic lymphohistiocytosis following enteric fever: A rare autopsy case reporten_US
dc.typeJournal Articleen_US
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