A Newborn With Familial Hemophagocytic Lymphohistiocytosis Complicated With Transfusion Associated Graft Versus Host Disease


Ozdemir A. , GÜNEŞ T. , CHIANG S. C. C. , ÜNAL E.

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.39, no.6, 2017 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 39 Issue: 6
  • Publication Date: 2017
  • Doi Number: 10.1097/mph.0000000000000777
  • Title of Journal : JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is characterized by activation of cytotoxic T and natural killer (NK) cells, and macrophages related to a spectrum of hyperinflammatory disorders. The clinical findings mainly include high fever, cytopenia, splenomegaly, phagocytosis, and proliferation of histiocytes in lymphoreticular tissue. To the best of our knowledge, transfusion-associated graft versus host disease (TA-GVHD) in a 13-day old male newborn with HLH is being reported first time in the literature. The aim of this report was to emphasize the importance of blood products irradiation in the prevention of the development of graft versus host disease especially among high-risk subjects such as newborns with HLH.