Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score.

Tesch V., Abolhassani H., Shadur B., Zobel J., Mareika Y., Sharapova S., ...Daha Fazla

The Journal of allergy and clinical immunology, cilt.145, ss.1452-1463, 2020 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 145
  • Basım Tarihi: 2020
  • Doi Numarası: 10.1016/j.jaci.2019.12.896
  • Dergi Adı: The Journal of allergy and clinical immunology
  • Sayfa Sayıları: ss.1452-1463


Background: Recent findings strongly support hematopoietic stem cell transplantation (HSCT) in patients with severe presentation of LPS-responsive beige-like anchor protein (LRBA) deficiency, but long-term follow-up and survival data beyond previous patient reports or meta-reviews are scarce for those patients who do not receive a transplant.