Random Skin Biopsy Is a Useful Procedure in the Evaluation of Hemophagocytic Lymphohistiocytosis: A Case Report and Review of Literature. (27th December 2022)

• Record Type: Journal Article
• Authors: Hegazy, Shaymaa; Moesch, John; Guerrero, Angela; Ho, Jonhan; Karunamurthy, Arivarasan
• Is Part Of: American journal of dermatopathology. Volume 44:Number 12(2022)
• Page Start: 925
• Page End: 928
• Publication Date: 2022-12-27
• Subjects: HLH -- hemophagocytosis -- M-HLH -- random skin biopsy; Skin -- Diseases -- Periodicals; Histology, Pathological -- Periodicals; 616.50705
• DOI: 10.1097/DAD.0000000000002301
• Languages: English
• ISSNs: 0193-1091

Abstract

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome, characterized by aberrant activation of T lymphocytes and macrophages leading to hypercytokinemia. HLH can be familial or a result of various secondary etiologies. We present a case of a 46-year-old woman with a past medical history of multiple sclerosis on rituximab who presented as a transfer from an outside hospital with numerous clinical abnormalities including recurrent episodes of fever of unknown origin for 3 weeks, persistent leukocytosis, hypertriglyceridemia, and steatohepatitis. Given the uncertain nature of her illness, she underwent a random skin biopsy from the abdominal region to exclude hematolymphoid malignancy. Histopathology revealed a brisk histiocytic rich dermal infiltrate accompanied by perivascular lymphocytic infiltrate. The histiocytes were enlarged and positive for muraminadase and CD68 stains exhibiting hemophagocytosis focally. As per the HLH-2004 protocol, our patient met the diagnostic criteria of HLH. Concurrent bone marrow biopsy revealed similar rare hemophagocytosis. Cytogenetics and molecular studies were negative, supporting secondary HLH.