[Autoimmune hematologic diseases in adult patients with common variable immunodeficiency].

BACKGROUND

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by defective immunoglobulin production. Twenty percent of patients may also have associated autoimmune diseases, especially hematologic, whose prevalence is estimated at 5 to 8%.

OBJECTIVE

To report the occurrence of autoimmune haematological disorders in CVID adults.

METHODS

We included 18 patients with CVID, who underwent hematologic evaluation, which included: CBC, serum folate or vitamin B complex and bone marrow aspirate.

RESULTS

Of a total of 18 patients, 11 women (61%), mean age 38 ± 13.5, six patients (33.3%) had autoimmune diseases, five of them had blood diseases (4 with PTI and one with Evans syndrome). Three patients had more than one autoimmune disease and one presented three autoimmune diseases (Evans syndrome, hypoparathyroidism and celiac disease with a history of autoimmune hemolytic anemia).

CONCLUSIONS

The prevalence of autoimmune diseases in this group of patients with CVID was higher than the 20% reported in the literature. Hematological diseases should be suspected in CVID patients, since half of our cases presented them.