| 特集名 | 全身性疾患における血液浄化療法 -- 急性期、および慢性期の対応 | |
|---|---|---|
| 題名 | ウイルス関連血球貪食症候群(virus associated HPS ; VAHPS)における血漿交換療法(PE) | |
| 発刊年月 | 2001年 03月 | |
| 著者 | 佐藤 寿伸 | 東北大学医学部附属病院血液浄化療法部 |
| 【 要旨 】 | ウイルス関連血球貪食症候群(VAHPS)は，サイトメガ口ウイルス(CMV)やEBウイルスなどの感染症に続いて，高熱，血球減少，高フェリチン血症などの異常を呈し，骨髄における血球貪食像を特徴とする症候群である．本稿では自験CMV-AHPSを提示し，その臨床経過と血漿交換療法(PE)を含む治療法について概説する．VAHPSは現在のところその発症機序が完全には解明されておらず，治療法に関する統一された見解も示されていないが，臨床の場では，その治療として経験的に効果が期待されるPEやgamma-グロブリン療法，ステロイド療法，化学療法などが行われることがある．しかし，その発症初期における予後規定因子が明らかではない現状では，大量の血漿成分を用いるPEを含めたこれら治療法の選択は，十分なインフォームド・コンセントに基づいて行われる必要がある． | |
| Theme | Management of Systemic Diseases with Blood Purification : Aute and Chronic Stage of Diseases | |
|---|---|---|
| Title | Plasmapheresis in virus associated hemophagocytic syndrome (VAHPS) | |
| 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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