Abstract

Objective: Hemophagocytic syndrome (HPS) is a severe complication of chronic rheumatic diseases particularly juvenile rheumatoid arthritis. But it is rarely described in Adult form of rheumatoid polyarthritis. We report a new case of HS complicating a seropositive rheumatoid polyarthritis. Case report: A 50- year old female with past history of a seropositive rheumatoid polyarthritis treated by corticosteroids and methotrexate, was admitted for deterioration of general status. She complained of fever and polyarthralgia. On physical examination, she had polyathritis and an hepato-splenomegaly. Routine blood analysis showed an increase of inflammatory indices with a pancytopenia, high levels of lactico-deshydrogenases and hepatic cytolysis. Intravascular disseminated coagulation was diagnosed. High levels of triglyceridemia and an hyperferritinaemia were noted. An hemophagocytic syndrome was suspected and cytological examination of bone marrow confirmed hemophagocytosis. Other aetiologies of HPS (immunodeficiency, infections, hemopathies, cancers and auto-immune diseases) were eliminated. We retained the diagnosis of HPS complicating a seropositive rheumatoid polyarthritis. The outcome was rapidly fatal with acute hepatocellular failure leading to death. Conclusion: Hemophagocytic syndrome is rarely reported with rheumatoid polyarthritis. In front of pancytopenia, it is important to be aware of this syndrome because its occurrence is associated with a poor prognosis and its optimal treatment has not been fully defined.

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