Liver disease predicts mortality in patients with XHIM but can be prevented by early hematopoietic stem cell transplantation.

Vian Azzu,Lucinda Kennard,Beatriz Morillo-Gutierrez,Mary Slatter,J. David M. Edgar,Dinakantha S. Kumararatne,William J.H. Griffiths

Journal of Allergy and Clinical Immunology（2018）

引用 17|浏览15

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摘要

In the largest detailed study of liver disease in X-linked immunodeficiency with hyper-IgM, we show that liver disease is commoner than generally recognized and that early hematopoietic stem cell transplantation may prevent liver disease progression and improve survival.

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关键词

CD40 ligand deficiency,XHIM,abnormal liver function,chronic hepatitis,hematopoietic stem cell transplant,sclerosing cholangitis

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