Abstract
IntroductionHemophagocytic lymphohistiocytosis is an immune-mediated syndrome that typically has a rapidly progressive course that can result in pancytopenia, coagulopathy, multi-system organ failure and death.Case presentationA 57-year-old Caucasian woman was referred in fulminant hemophagocytic lymphohistiocytosis, with fever, pancytopenia, splenomegaly, mental status changes and respiratory failure. She was found to have stage IV classical Hodgkin lymphoma, in addition to Epstein-Barr virus and cytomegalovirus viremia. Her presentation was preceded by a 3-year prodrome consisting of cytopenia and fever that were partially controlled by steroids and azathioprine.ConclusionFulminant hemophagocytic lymphohistiocytosis may follow a prodromal phase that possesses features suggestive of a chronic form of hemophagocytic lymphohistiocytosis, but which may also resemble immune cytopenias of other causes. A diagnosis of hemophagocytic lymphohistiocytosis should be considered in the setting of chronic pancytopenia.
Highlights
Hemophagocytic lymphohistiocytosis is an immune-mediated syndrome that typically has a rapidly progressive course that can result in pancytopenia, coagulopathy, multisystem organ failure and death
A diagnosis of hemophagocytic lymphohistiocytosis should be considered in the setting of chronic pancytopenia
We have described a case of acquired hemophagocytic lymphohistiocytosis (HLH) that presented in a fulminant form following a 3-year prodrome that was consistent with a mild, chronic form of HLH
Summary
We have described a case of acquired HLH that presented in a fulminant form following a 3-year prodrome that was consistent with a mild, chronic form of HLH. ALT: alanine aminotransferase; ANA: antinuclear antibody; AST: aspartate aminotransferase; BiPAP: bilevel positive airway pressure; CMV: cytomegalovirus; CT: computed tomography; EBV: Epstein-Barr virus; GI: gastrointestinal; Hb: hemoglobin; HHV-6: human herpesvirus 6; HIV: human immunodeficiency virus; HL: Hodgkin Lymphoma; HLH: hemophagocytic lymphohistiocytosis; LMP: latent membrane protein; MCV: mean cell volume; RBC: red blood cell; RS: Reed-Sternberg cell; sCD25: soluble CD25; WBC: white blood cell count. Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal
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