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MR Vol.14 No.3 indexに戻る
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MODERN RHEUMATOLOGY
Vol.14 No.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 |
| 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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