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MODERN RHEUMATOLOGY Vol.14 No.3
>MR14-3
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| Autoimmune-associated hemophagocytic syndrome |
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| Shunichi Kumakura1 , Hiroto Ishikura2, Masahiro Kondo3, Yohko Murakawa3, Junichi Masuda1 and Shotai Kobayashi3 |
(1) Division of Blood Transfusion, School of Medicine, Shimane University, Izumo, 693-8501, Japan
(2) Department of Clinical Nursing, Shimane University, Izumo, Japan
(3) Department of Neurology, Hematology & Rheumatology, School of Medicine, Shimane University, Izumo, Japan |
Received: 28 March 2003 Accepted: 16 February 2004 |
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Abstract |
| Hemophagocytic syndrome (HPS) is a clinicopathological condition characterized by the activation of histiocytes with prominent hemophagocytosis in bone marrow and other reticuloendothelial systems. The occurrence of HPS is usually associated with underlying disorders such as infection and lymphoma. Recently, we described patients with autoimmune disease who developed HPS. In these cases there was no evidence of underlying infection and malignancy, and the occurrences of HPS were associated with active autoimmune disease. Based on these observations, we described autoimmune-associated hemophagocytic syndrome (AAHS). This disease entity is becoming better known, and case reports presenting features compatible with clinical AAHS are increasing. Here, we review the clinical aspects, mechanisms, diagnosis, and treatment of AAHS according to our data and that in the literature. |
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| Key words |
| Autoimmune-associated hemophagocytic syndrome (AAHS) - Autoimmune disease - Hemophagocytic syndrome (HPS) - Hemophagocytosis |
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