Bone marrow transplantation for CVID-like humoral immune deficiency associated with red cell aplasia.
Patients with common variable immunodeficiency (CVID) have a higher incidence of autoimmune disease, which may mark the disease onset; however, anemia secondary to pure red cell aplasia is an uncommon presenting feature. Here, we describe a case of CVID-like humoral immune deficiency in a child who initially presented with red cell aplasia and ultimately developed progressive bone marrow failure. Although bone marrow transplantation (BMT) has been associated with high mortality in CVID, our patient was successfully treated with a matched sibling BMT and engrafted with >98% donor chimerism and the development of normal antibody titers to diphtheria and tetanus toxoids.
UF Brain Tumor Immunotherapy Program, Preston A. Wells Jr. Center for Brain Tumor Therapy, McKnight Brain Institute, Department of Neurosurgery, University of Florida, Gainesville, Florida.|Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Florida, Gainesville, Florida.
Elias J
EJ
Sayour
Departments of Internal Medicine and Pediatrics, Wake Forest University School of Medicine, Winston Salem, North Carolina.|Division of Allergy-Immunology, Department of Pediatrics, and Department of Immunology, Duke University Medical Center, Durham, North Carolina.
Talal
T
Mousallem
Division of Pediatric Hematology-Oncology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
David
D
Van Mater
Department of Patholgy, Duke University Medical Center, Durham, North Carolina.
Endi
E
Wang
Division of Pediatric Bone Marrow Transplantation, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
Paul
P
Martin
Division of Allergy-Immunology, Department of Pediatrics, and Department of Immunology, Duke University Medical Center, Durham, North Carolina.
Rebecca H
RH
Buckley
Division of Pediatric Hematology-Oncology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina.
Raymond C
RC
Barfield
D016026:Bone Marrow Transplantation; D002648:Child; D017074:Common Variable Immunodeficiency / Q000276:immunology / Q000628:therapy*; D006801:Humans; D056724:Immunity, Humoral; D008297:Male; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000276:immunology
common variable immunodeficiency; pure red cell aplasia; transplantation