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

European Medical Journal Hematology

Warda Fatmi¹  Narmin Khan¹  Zehra Iqbal¹  Muhammad Sohaib Asghar¹  Abubakar Tauseef¹  Maryam Zafar¹  Uzma Rasheed²  Basmah Fayaz²  Mohammed Akram²  Nimra Shaikh² 

[1] Internal Medicine Department, Dow University Hospital, Dow University of Health Sciences, Karachi, Pakistan;Internal Medicine Department, Liaquat National Hospital and Medical College, Karachi, Pakistan;

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

DOI  :  

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown