| Theme | Management of Systemic Diseases with Blood Purification : Aute and Chronic Stage of Diseases | |
|---|---|---|
| Title | Plasmapheresis in virus associated hemophagocytic syndrome (VAHPS) | |
| Publish Date | 2001/03 | |
| Author | Toshinobu Sato | Department of Blood Purification, Tohoku University School of Medicine |
| [ Summary ] | Virus associated hemohagocytic syndrome (VAHPS) is a disease entity associated with active viral infection such as cytomegalovirus (CMV), Epstein Barr virus and others. Clinically, this syndrome is characterized by high fever, peripheral blood cytopenia including at least two cell lines, high levels of serum ferritin and serum LDH, as well as liver dysfunction. In addition, VAHPS essentially shows histiocytic hyperplasia, with prominent hemophagocytosis in bone marrow or spleen. In this section, a case of VAHPS associated with CMV infection is presented and the therapy for VAHPS, including plasmapheresis (PE) is briefly reviewed. Unfortunately, the details of the pathogenesis of VAHPS are unknown at present, although recent studies have suggested that hypercytokinemia is involved in VAHPS. In general, the prognosis for VAHPS is not always good. In fact, some cases are progressive and fatal. Therefore, PE is performed to remove hypercytokinemia in certain cases. Alternatively, high doses of gamma-globulin, steroids or cytotoxic drugs are used to control abnormally activated T cells or macrophages. Since all the treatments for HAHPS descried above are still empirical and because the prognostic factors of VAHPS at the early phase are not yet clear, it is very important that treatment of VAHPS should be performed with adequate informed consent. Particularly, the physician should explain the risks of PE, using a large amount of plasma, to the patient and their family. |
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