title: RHD zygosity predicts degree of platelet response to anti-D immune globulin treatment in children with immune thrombocytopenia.
abstract: Anti-D immunoglobulin is a common front-line treatment for childhood immune thrombocytopenia (ITP) that typically results in a rapid and significant increase in platelet count. Unpredictable treatment responses and interpatient variability limit more widespread use. We hypothesized that anti-D response variability is influenced by RHD gene zygosity and erythrocyte D antigen expression. We compared RHD zygosity and quantitative D antigen expression to anti-D treatment results. Hemizygous RHD subjects demonstrated significantly higher platelet increases and peak platelet counts than homozygous RHD subjects. Future studies should investigate the mechanisms by which RHD zygosity and D antigen expression affect platelet responses to anti-D immunoglobulin.
authors.affiliation: Texas Children's Hematology Center, Houston, TX 77030, USA. jmdespot@txch.org
authors.forename: Jenny M
authors.initials: JM
authors.lastname: Despotovic
authors.forename: Patrick T
authors.initials: PT
authors.lastname: McGann
authors.forename: Matthew
authors.initials: M
authors.lastname: Smeltzer
authors.forename: Banu
authors.initials: B
authors.lastname: Aygun
authors.forename: Russell E
authors.initials: RE
authors.lastname: Ware
mesh_terms: D001792:Blood Platelets / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D005786:Gene Expression Regulation / Q000187:drug effects*; D057898:Hemizygote; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage*; D007223:Infant; D008297:Male; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000188:drug therapy / Q000235:genetics; D012189:Retrospective Studies; D012204:Rh-Hr Blood-Group System / Q000096:biosynthesis / Q000235:genetics; D018029:Rho(D) Immune Globulin / Q000008:administration & dosage*
chemical_list: D007155:Immunologic Factors; D012204:Rh-Hr Blood-Group System; D018029:Rho(D) Immune Globulin; C026410:Rho(D) antigen
keywords: RHD gene; anti-D immune globulin; hemolysis; immune thrombocytopenia