Adult-Onset Still’s Disease Complicated with Haemophagocytic Lymphohistiocytosis (HLH): A Case Report

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially aggressive and life-threatening syndrome of overactive histiocytes and lymphocytes that commonly affects infants; it is also observed in children and adults of all ages. The disease is differentiated into either primary or secondary causes. Primary HLH tends to be of genetic origin, while secondary HLH results from either infection, autoimmune disorders, or malignancies. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. This paper presents a case of HLH in a tertiary care hospital, associated with adult-onset Still’s disease, diagnosed on both biochemical criteria and histopathologic examination of bone marrow smear.

Keywords

• adult-onset still’s disease
• autoimmune
• connective tissue
• haematology
• haemophagocytic lymphohistiocytosis (hlh)
• immunology
• infectious diseases
• macrophage activation syndrome
• rheumatology