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Lethal graft‐versus‐host disease in congenital neutropenia caused by p14 deficiency after allogeneic bone marrow transplantation from an HLA‐identical sibling
Author(s) -
Bohn Georg,
HardtkeWolenski Matthias,
Zeidler Cornelia,
Maecker Britta,
Sauer Martin,
Sykora KarlWalter,
Grigull Lorenz,
Welte Karl,
Klein Christoph
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21643
Subject(s) - medicine , immunology , sibling , neutropenia , human leukocyte antigen , bone marrow , tumor necrosis factor alpha , congenital neutropenia , graft versus host disease , microchimerism , transplantation , disease , antigen , chemotherapy , fetus , psychology , pregnancy , developmental psychology , biology , genetics
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‐) α production upon toll‐like receptor stimulation. We hypothesize that p14 deficiency predisposes to GvHD through increased TNF‐α production. Adequate genetic testing is needed to prospectively assess potential risk factors for GvHD in defined SCN subgroups. Pediatr Blood Cancer 2008;51:436–438. © 2008 Wiley‐Liss, Inc.