Hemophagocytic syndrome

Kumakura Shunichi

doi:10.14961/cra.30.241

Hemophagocytic syndrome（HPS）is a severe and life-threatening disease. Characteristic manifestations of HPS include fever, hepatosplenomegaly, pancytopenia, coagulopathy, and liver dysfunction. HPS is classified according to the underlying etiology into either primary（genetic）or secondary（reactive）HPS. Autoimmune-associated hemophagocytic syndrome（AAHS）is a secondary HPS, which is associated with underling autoimmune disease. There are currently no validated diagnostic criteria for AAHS, but several criteria have been proposed to date. These criteria pose particular problems for the diagnosis of AAHS, because some clinical and laboratory criteria, such as fever, may be presented in the underlying autoimmune disease itself. It is therefore necessary to establish validated diagnostic criteria. Corticosteroids were most commonly used for patients with AAHS, and 58% of patients were responded. Patients refractory to initial corticosteroids were usually treated by cyclosporine, intravenous cyclophosphamide（IVCY）or intravenous immunoglobulin G（IVIG）, and among these therapies IVCY was highly effective. Treatment with biologic agents resulted in favorable effects in the majority of patients. The development of a new approach using biologic agents, as well as small molecule inhibitors of intracellular signal transduction pathways, in the treatment of AAHS, seems to be challenging.

Hemophagocytic syndrome

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