Assessment of low immunoglobulin levels and clinical manifestations in patients with mastocytosis

BACKGROUND: Patients with a low IgG level alone or with low IgA or IgM levels have been reported to be susceptible to respiratory tract infections and recurrent sinusitis. Patients diagnosed with common variable immunodeficiency 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. OBJECTIVE: We sought to determine the distribution of immunoglobulin levels in children and adults with mastocytosis, as well as to evaluate the impact of low immunoglobulin levels on the clinical management of patients with mastocytosis. METHODS: We performed a 10-year retrospective analysis on 320 adult and pediatric patients with mastocytosis for immunoglobulin levels by using an electronic medical query. We identified 25 adults and 9 children with 1 or more low immunoglobulin levels. Patient records were examined for a history of infections and autoimmune disorders. RESULTS: Serum immunoglobulin levels in children and adults with mastocytosis fell within a normal range. Of the patients with low IgG levels alone or with low IgM and/or IgA levels, 20% had a history of infections and 20% of adults had autoimmune disorders. The most common infection was recurrent otitis media. CONCLUSION: Patients with mastocytosis typically have normal immunoglobulin levels. With few exceptions, those with low immunoglobulin levels did not have frequent infections or autoimmune diseases. These data support the conclusion that routine determination of immunoglobulin levels in patients with mastocytosis is not required and should be reserved for patients with clinical conditions that might be related to an immunoglobulin deficiency.