Major Histocompatibility Complex (MHC) II Deficiency Needs Early Stem Cell Transplantation: A Case Report
PDF

Major Histocompatibility Complex (MHC) II Deficiency Needs Early Stem Cell Transplantation: A Case Report

Anatol J Gen Med Res 2007;17(1):51-55 DOI: 10.5222/terh.2007.44194
, , , ,
PDF
Permissions

Abstract

Major histocompatibility complex (MHC) II deficiency is a rare primary immunodeficiency disorder characterized by defects in human leukocyte antigen class II expression and lack of cellular and humoral immune responses to foreign antigens. Clinical onset occurs early in life with recurrent infeetions and chronic diarrhea. The prognosis is poor and only curative treatment is stem cell transplantation (SCT) performed early in life. In t his report, we present a patient who had immune deficiency caused by MHC Class II expression defect. He died because of sepsis and multi-organ failure as stem cel transplantation could not be performed immediately after diagnosis.

Keywords: MHC Class II, immune deficiency, stem cell transplantation