Autosomal Recessive Chronic Granulomatous Disease, IgA Deficiency and Refractory Autoimmune Thrombocytopenia Responding to Anti-CD20 Monoclonal Antibody

Immunodeficiency and autoimmune disease may occur concomitantly in the sameindividual. some of the immunodeficiency syndromes, especially humoral defects areassociated with autoimmune disorders. hematological manifestations such asthrombocytopenia and hemolytic anemia are the most common presentations. persistentantigen stimulation due to an inherent defect in the ability of the immune system to eradicatepathogens is the primary cause leading to autoimmunity in patients with primaryimmunodeficiency states.we describe a 10 year old iranian girl with chronic granulomatous disease -the autosomalrecessive type with mutation of ncf1 gene p47- associated with selective iga deficiency,refractory immune thrombocytopenia that showed an excellent response to rituximab (anti-cd20 monoclonal antibody).patients with primary immunodeficiencies may have variable autoimmune manifestations.so for early detection and appropriate treatment, autoimmune diseases should always besuspected in such patients.