title: Presence of Idiopathic Thrombocytopenic Purpura and autoimmune hemolytic anemia in the patients with common variable immunodeficiency.
abstract: Common Variable Immunodeficiency (CVID) is a heterogeneous group of disorders characterized by hypogammaglobulinemia and an increased susceptibility to recurrent infections as well as autoimmunity and malignancies. Idiopathic Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) are two autoimmune disorders which may be seen in association with CVID. Among 85 CVID patients, seven cases had ITP and/or AIHA (8%). Four of these patients had one or more episodes of ITP, one patient had AIHA, and two patients had both ITP and AIHA (Evans syndrome). Almost, all patients experienced chronic and recurrent infections mostly in respiratory and gastrointestinal systems during the course of the disease. Among the seven patients, five presented their underlying disease with recurrent respiratory and/or gastrointestinal tract infections, while in two remaining patients, CVID was presented with ITP. Three patients died until now; two because of hepatic failure and one due to pulmonary hemorrhage. As CVID is prone to autoimmune disorders, it should be considered as a differential diagnosis of adult-onset ITP and possibly in children. Chronic and recurrent ITP, especially in the presence of propensity to respiratory and gastrointestinal infections mandate the evaluation for an underlying immune dysregulation such as CVID.
authors.affiliation: Department of Pediatrics, Division of Hematology, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Ramyar
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Kasra
authors.initials: K
authors.lastname: Moazzami
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Mehdi
authors.initials: M
authors.lastname: Yeganeh
authors.forename: Taher
authors.initials: T
authors.lastname: Cheraghi
authors.forename: Nima
authors.initials: N
authors.lastname: Pouladi
authors.forename: Golnaz
authors.initials: G
authors.lastname: Heydari
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.forename: Ali Akbar
authors.initials: AA
authors.lastname: Amirzargar
authors.forename: Nima
authors.initials: N
authors.lastname: Parvaneh
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D003967:Diarrhea; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007136:Immunoglobulins / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007492:Iran; D008297:Male; D008875:Middle Aged; D010033:Otitis Media; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D012008:Recurrence; D012189:Retrospective Studies; D012852:Sinusitis
chemical_list: D007136:Immunoglobulins; D016756:Immunoglobulins, Intravenous