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Bone marrow transplantation for CVID‐like humoral immune deficiency associated with red cell aplasia
Author(s) -
Sayour Elias J.,
Mousallem Talal,
Mater David,
Wang Endi,
Martin Paul,
Buckley Rebecca H.,
Barfield Raymond C.
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26092
Subject(s) - medicine , pure red cell aplasia , common variable immunodeficiency , immunology , aplasia , immunodeficiency , bone marrow aplasia , bone marrow , immune system , transplantation , antibody
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.