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Ruxolitinib as Adjunctive Therapy for Hemophagocytic LymPhohistiocytosis after Liver Transplantation: A Case Report and Literature Review.


ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal hyperinflammatory disorder characterized by dysfunctional cytotoxic T and natural killer cells. Liver transplantation is a predisposing factor for HLH. High mortality rates were reported in 40 cases of HLH following liver transplantation in adults and children. Herein, we describe a case of adult HLH triggered by cytomegalovirus (CMV) infection shortly after liver transplantation. The patient was successfully treated with ruxolitinib combined with a modified HLH-2004 treatment strategy. Our case is the first to report the successful use of ruxolitinib with a modified HLH-2004 strategy to treat HLH in a solid organ transplantation recipient.

SUBMITTER: He K 

PROVIDER: S-EPMC9656798 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature

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Ruxolitinib as Adjunctive Therapy for Hemophagocytic LymPhohistiocytosis after Liver Transplantation: A Case Report and Literature Review.

He Kang K   Xu Shanshan S   Shen Lijing L   Chen Xiaosong X   Xia Qiang Q   Qian Yongbing Y  

Journal of clinical medicine 20221026 21


Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal hyperinflammatory disorder characterized by dysfunctional cytotoxic T and natural killer cells. Liver transplantation is a predisposing factor for HLH. High mortality rates were reported in 40 cases of HLH following liver transplantation in adults and children. Herein, we describe a case of adult HLH triggered by cytomegalovirus (CMV) infection shortly after liver transplantation. The patient was successfully treated with r  ...[more]

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