Development of common variable immunodeficiency in a patient with Evans syndrome treated by autologous stem cell transplantation

• Published in: Pediatric allergy and immunology Vol. 14; no. 4; pp. 334 - 337
• Main Authors: STARY, Jan, SEDLACEK, Petr, VODVARKOVA, Sarka, GASOVA, Zdenka, BARTUNKOVA, Jirina
• Format: Journal Article
• Language: English
• Published: Oxford, UK Munksgaard International Publishers 01.08.2003; Blackwell
• Subjects: Anemia, Hemolytic, Autoimmune - immunology; Anemia, Hemolytic, Autoimmune - therapy; autologous stem cell transplantation; Biological and medical sciences; C4 deficiency; Child; common variable immunodeficiency; Common Variable Immunodeficiency - etiology; Common Variable Immunodeficiency - immunology; CVID; Dermatitis Herpetiformis - immunology; Dermatitis Herpetiformis - therapy; Evans syndrome; Fundamental and applied biological sciences. Psychology; Fundamental immunology; General aspects; Humans; Immunoglobulin G - immunology; Male; Medical sciences; Neutropenia - immunology; Neutropenia - therapy; Postoperative Complications - etiology; Postoperative Complications - immunology; Purpura, Thrombocytopenic, Idiopathic - immunology; Purpura, Thrombocytopenic, Idiopathic - therapy; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Stem Cell Transplantation; Syndrome; T-Lymphocytes - immunology; T-Lymphocytes - transplantation; Transplantation, Autologous; Human; Immunopathology; Pediatrics; Common variable immunodeficiency; Stem cell; Deficiency; autologous stem cell transplantation; Hematopoietic cell; Autoimmune disease; Patient; Hemopathy; C4 deficiency; Evans syndrome; CVID; Autograft; Platelet; Immunology; Treatment; Development; Graft
• ISSN: 0905-6157; 1399-3038
• DOI: 10.1034/j.1399-3038.2003.00034.x

We describe a case report of a patient who developed common variable immunodeficiency (CVID) after autologous haematopoietic stem cell transplantation (SCT) for recurrent Evans syndrome. The disease manifested as attacks of haemolytic anaemia, thrombocytopenia and neutropenia from the age of 12 years. Presence of autoantibodies to blood elements was confirmed together with C4 deficiency. The patient also suffered from dermatitis herpetiformis Duhring without signs of coeliac disease. Autologous T cell‐depleted peripheral blood stem cell (PBSC) transplant following conditioning regimen was performed at the age of 20 years. Immunological reconstitution was incomplete and 2 years after SCT he fulfilled laboratory criteria for common variable immunodeficiency (CVID). The patient was found to be a carrier of a risk haplotype for development of CVID DRB1*03/DQB1*0201. We conclude that T cell‐depleted SCT here performed for autoimmune manifestations can hasten development of CVID in genetically predisposed patients.