An Interesting Case of Hemophagocytic Lymphohistiocytosis Secondary to Systemic Lupus Erythematosus

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a reactive condition marked by cytopenias and clinical features of systemic inflammation related to macrophage activation. It may be familial and may present early in life or sporadic and may affect people of any age. HLH secondary to systemic lupus erythematosus (SLE) is a rare clinical entity with an estimated prevalence of 0.9–4.6%. A 19-year-old man with no significant past medical history presented with complaints of high-grade fever with chills and bleeding manifestations, such as hematemesis, hemoptysis, epistaxis, and petechial rash in both upper limbs and lower limbs. Laboratory investigations showed significant pancytopenia and raised plasma levels of ferritin, triglycerides, and soluble interleukin (IL)-2. Ultrasonography of the abdomen showed mild hepatomegaly. A bone marrow biopsy showed evidence of mild hemophagocytosis. Antinuclear antibody (ANA) by indirect immunofluorescence assay (IFA) and double-strand deoxyribonucleic acid (dsDNA) came positive, and a diagnosis of HLH secondary to SLE was made. The patient was treated symptomatically with PCV and random donor platelet (RDP) transfusions and definitively with steroid therapy. HLH provokes potential life-threatening systemic toxicity and organ failure. Thus, prompt recognition when a patient presents with unexplained fever, cytopenias, and hepatosplenomegaly is required for timely treatment to prevent end-organ irreversible damage.

Keywords

• cytopenia
• hemophagocytic lymphohistiocytosis
• immunological activation
• systemic lupus erythematosus