Lethal graft-versus-host disease in congenital neutropenia caused by p14 deficiency after allogeneic bone marrow transplantation from an HLA-identical sibling

被引：5

作者：

Bohn, Georg ^{[1
]}

Hardtke-Wolenski, Matthias ^{[1
]}

Zeidler, Cornelia ^{[1
]}

Maecker, Britta ^{[1
]}

Sauer, Martin ^{[1
]}

Sykora, Karl-Walter ^{[1
]}

Grigull, Lorenz ^{[1
]}

Welte, Karl ^{[1
]}

Klein, Christoph ^{[1
]}

机构：

[1] Hannover Med Sch, Dept Pediat Hematol & Oncol, D-30625 Hannover, Germany

来源：

PEDIATRIC BLOOD & CANCER | 2008年 / 51卷 / 03期

关键词：

congenital neutropenia; GvHD; HLA-identical transplantation; immunodeficiency; p14;

D O I：

10.1002/pbc.21643

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

The molecular heterogeneity of severe congenital neutropenia (SCN) is increasingly recognized and may influence the risk-benefit assessment of therapeutic strategies. We report on a patient with p14 deficiency who succumbed to severe grade IV graft-versus-host disease (GvHD) after a human leukocyte antigen-identical bone marrow transplantion (BMT) from a sibling donor. Before BMT, in vitro generated p14-deficient dendritic cells showed a markedly elevated tumor necrosis factor (TNF-) alpha production upon toll-like receptor stimulation. We hypothesize that p14 deficiency predisposes to GvHD through increased TNF-alpha production. Adequate genetic testing is needed to prospectively assess potential risk factors for GvHD in defined SCN subgroups.

引用

页码：436 / 438

页数：3

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