Systemic lupus erythematosus associated hemophagocytic lymphohistiocytosis: A case report and literature review

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune overstimulation. HLH commonly manifests as multiple organ failure without apparent physiological stress. Primary HLH relates to genetic defects, whereas secondary HLH is triggered by infection, malignancy, or autoimmune disease. An aberrant natural killer cell or absence of T lymphocyte apoptosis causes uncontrolled inflammation. Secondary HLH in patients with systemic lupus erythematosus (SLE) is uncommon with an estimated prevalence of 0.9%. This report describes a male with SLE associated secondary HLH. Patients affected by HLH require prompt management, but delayed diagnosis is common due to its variable presentation. An eight week induction therapy with dexamethasone, etoposide, and intrathecal methotrexate increases survival in primary HLH and virus-associated secondary HLH. However, no standardized regimen exists for autoimmune associated HLH. Some patients respond to steroid monotherapy, while refractory patients may require cyclosporin, cyclophosphamide, or tacrolimus.