Systemic Lupus Erythematosus Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review

• DOI: 10.11648/j.ajim.20140203.13
• Publication Date: 2014-09-02T06:11:41Z
• Authors: Po-Chung Cheng
• Keywords: Hemophagocytic Lymphohistiocytosis; Hemophagocytosis

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