Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome involving excessive immune activation. It can be primary (familial) or secondary (triggered by infection, malignancy, or rheumatological disease). This is a case of a previously healthy 43-year-old African American woman who presented with fever and confusion. The patient was eventually diagnosed with pulmonary aspergillosis and responded well to antifungal therapy. She met the diagnostic criteria of HLH-2004 trial for hemophagocytic lymphohistiocytosis. She also fulfilled the 2019 classification criteria for systemic lupus erythematosus (SLE) without the classical signs and symptoms of SLE. HLH management includes supportive management, treatment of underlying condition, and immunosuppressive treatment. Etoposide and dexamethasone are commonly used treatments for HLH; however, underlying active infection can limit the treatment options. In our case, the patient was treated with steroids and hydroxychloroquine. Her condition gradually improved and she recovered without complications. Based on our literature review, we encountered six cases of HLH secondary to Aspergillosis with a mean age of approximately 47 years. The diagnosis of HLH is often delayed because of nonspecific presentation. Early identification and treatment are crucial to improve the survival rate.
Keywords: hemophagocytic lymphohistiocytosis (hlh); immune-mediated inflammatory disorder; neurological signs and symptoms; pulmonary aspergillosis; secondary hlh.
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