Hyperimmunglobulin M Syndrome


Gülcan Ü., ÇEKİÇ Ş. , Gültekin Sara K.

GUNCEL PEDIATRI-JOURNAL OF CURRENT PEDIATRICS, vol.12, no.2, pp.81-87, 2014 (Journal Indexed in ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 12 Issue: 2
  • Publication Date: 2014
  • Doi Number: 10.4274/jcp.18894
  • Title of Journal : GUNCEL PEDIATRI-JOURNAL OF CURRENT PEDIATRICS
  • Page Numbers: pp.81-87
  • Keywords: Hyper IgM syndrome, immune deficiency, IVIG

Abstract

Hyperimmunoglobulin M syndrome is a primary immune deficiency characterized by increased or normal levels of serum IgM with clearly decreased serum IgG, IgA and IgE levels. Patients affected with hiper IgM syndrome are susceptible to recurrent pyogenic infections, to autoimmune diseases induced by IgM antibodies and to malignant lymphoproliferative disease IgM producing B cells. Intravenous immunoglobulin replacement for therapy every 3-4 weeks is effective. Prophylactic treatment for P. jirovecii on trimethoprim-sulfhamethoxazole, in selected neutropenic patients granulocyte colony stimulated factor (G-CSF) treatment is suggested. Bone marrow or cord blood transplantation may provide cure for this syndrome.