Hyperimmunglobulin M Syndrome

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.