Three Cases of Severe Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (EBV-HLH) Successfully Treated with HLH-94 Protocol-Based Immunochemotherapy.
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EBV-HLH is characterized by a rapidly deteriorating clinical course, caused by uncontrolled severe hypercytokinemia. In recent years, the international HLH-94 protocol has begun to be used with success in the treatment of HLH ; however, there is no consensus on the best therapy for EBV-HLH. It has been widely proven that cyclosporin-A (CSA) efficiently and rapidly suppresses T cell function and lymphokine production. It seems rational to administer CSA in the early phase of EBV-HLH to suppress the cytokine storm caused by abnormally activated T cells and activated macrophages. In this report, we present three patients with severe EBV-HLH who were treated successfully with the HLH-94 protocol-based immunochemotherapy. One was treated with the HLH-94 protocol, and two patients who suffered from very severe tissue damage were treated simultaneously with the HLH-94 protocol and CSA (3 mg/kg/day, conti.div). All of them entered remission and have been well for more than 33 months. To evaluate the clinical effects of CSA, laboratory data were compared between cases. Hematopoietic recovery and the normalization of liver enzyme and ferritin level were faster in the two patients treated with HLH-94 protocol and CSA. We observed no adverse effects attributable to CSA. These observations suggest that the introduction of CSA along with HLH-94 protocol in the early clinical course has a beneficial effect, especially on patients with severe EBV-HLH.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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