Type II Bare Lymphocyte Syndrome: Role of Peripheral Blood Flow Cytometry and Utility of Stem Cell Transplant in Treatment

Michael E. Kallen, Sheeja T. Pullarkat

Research output: Contribution to journalArticlepeer-review

Abstract

Major histocompatibility complex class II (MHCII) deficiency is a rare autosomal recessive immunodeficiency disorder characterized by lack of expression of MHCII molecules, causing defective CD4+ lymphocyte function and an impaired immune response. Clinical manifestations include susceptibility to severe bacterial, viral, and fungal infections which can lead to failure to thrive and childhood death. The only definitive treatment to date is allogeneic stem cell transplantation. Here, we share our experience of 2 patients who presented with MHCII deficiency. We will discuss the role of diagnostic modalities and stem cell transplantation.

Original languageEnglish (US)
Pages (from-to)e245-e249
JournalJournal of Pediatric Hematology/Oncology
Volume37
Issue number4
DOIs
StatePublished - Dec 1 2015

Keywords

  • Bare Lymphocyte Syndrome
  • MHC II deficiency
  • stem cell transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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