Korean J Med.  2014 Mar;86(3):372-376. 10.3904/kjm.2014.86.3.372.

A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Rheumatoid Arthritis

• Choi IA
• Lee EY
• Lee EB
• Song YW

• KMID: 1867373
• DOI: http://doi.org/10.3904/kjm.2014.86.3.372

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a condition caused by excessive activation and expansion of T lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity. It is a life-threatening condition, and the reported mortality rates reach 20% to 30%. It is usually associated with infection, malignancy, or autoimmune disease, but rarely with rheumatoid arthritis (RA). We recently experienced a case of HLH with rapid progression resulting in mortality in a 38-year-old female patient with long-standing RA. She visited the clinic for evaluation of a common cold-like illness. She had hypotension, liver enzyme elevation, and pancytopenia. After admission, her hypotension continued and disseminated intravascular coagulation and metabolic acidosis developed and progressed with the appearance of azotemia. Despite supportive management, she died on the fifth hospital day. HLH should be considered as a differential diagnosis when patients with RA show acute illness with fever, cytopenia, hepatic failure, and coagulopathy.

Keyword

Lymphohistiocytosis; Hemophagocytic; Arthritis; Rheumatoid

MeSH Terms

Acidosis
Adult
Arthritis
Arthritis, Rheumatoid*
Autoimmune Diseases
Azotemia
Diagnosis, Differential
Disseminated Intravascular Coagulation
Female
Fever
Histiocytes
Humans
Hypotension
Liver
Liver Failure
Lymphohistiocytosis, Hemophagocytic*
Mortality
Pancytopenia
T-Lymphocytes