Hemophagocytic Lymphohistiocytosis and Clostridium difficile Infection: A Diagnostic Dilemma

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition that often goes underdiagnosed because of broad and non-specific symptomatology, usually consisting of fever, hepatosplenomegaly, and multiorgan failure. This disorder can be driven by genetic components (primary) or acquired (secondary) causes related to infectious, autoimmune, or malignant processes. HLH pathogenesis derives from overactive and dysregulated immune system responses. This disorder often goes misdiagnosed because of similar clinical and laboratory findings to septicemia. Cases of HLH most commonly coexist with Epstein-Barr virus (EBV). Clostridium difficile (C. difficile) infection causing HLH has also rarely been described in the literature. A firm knowledge of HLH association with clostridial infection is essential to recognize. A presumed diagnosis of HLH in a decompensating patient may prompt the initiation of appropriate treatment earlier and improve clinical outcomes. We discuss the diagnostic and management difficulties associated with these concurrent conditions.

Acute Liver Failure Caused by Secondary Hemophagocytic Lymphohistiocytosis After COVID-19 Vaccination – Case Report and Literature Review

“…To our best knowledge, three cases were described, exclusively in immunosuppressed patients (long-term corticosteroid therapy due to earlier HLH, NKT lymphoma, and ulcerative colitis therapy with 6-mercaptopurine). 22 Our patient was immunocompetent, which alongside a clinical and microbiological resolution of the C. difficile infection after vancomycin therapy, makes it unlikely C. difficile infection being provoking factor. A possible limitation of our case report is the absence of Epstein-Barr viremia confirmation in the context of urgent need for pretransplantation work-up.…”