Low Immunoglobulin Levels In Patients With Mastocytosis And Associated Clinical Manifestations

Abstract

Patients with low IgG alone or in combination with low IgA or IgM have been reported to be susceptible to respiratory tract infections and recurrent sinusitis. Patients with CVID also have a higher prevalence of autoimmune diseases and lymphoid malignancies. Mastocytosis is a myeloproliferative disease that is not typically associated with autoimmune disease or frequent infections. We determined the prevalence of infectious and autoimmune disorders in patients with mastocytosis with low IgG alone or in combination with low IgM and/or IgA to ascertain if there was clinical relevance. We performed a 10 year retrospective analysis on 320 adult and pediatric patients with all variants of mastocytosis using an electronic medical query. We identified 26 adults and 9 children with one or more low immunoglobulins levels. These patient records were then examined for a history of infections and autoimmune disorders. Overall, among these 35 patients, 17% of patients had a history of infections and 11% had an autoimmune disorder. The most common infectious condition was recurrent otitis media (OM). No patients required hospitalization for infection, two patients met criteria for CVID but did not require replacement therapy. Only one pediatric patient with low IgG received IgG replacement therapy for OM and mastoiditis. The routine determination of immunoglobulin levels in mastocytosis is thus not required and should be reserved for those with a clinical condition which might relate to a low immunoglobulin levels.