Presence of Idiopathic Thrombocytopenic Purpura and autoimmune hemolytic anemia in the patients with common variable immunodeficiency.
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.
Ramyar
Asghar
A
Department of Pediatrics, Division of Hematology, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Aghamohammadi
Asghar
A
Moazzami
Kasra
K
Rezaei
Nima
N
Yeganeh
Mehdi
M
Cheraghi
Taher
T
Pouladi
Nima
N
Heydari
Golnaz
G
Abolhassani
Hassan
H
Amirzargar
Ali Akbar
AA
Parvaneh
Nima
N
Moin
Mostafa
M
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
D007136:Immunoglobulins; D016756:Immunoglobulins, Intravenous