title: Detection and analysis of autoantigens targeted by autoantibodies in immunorelated pancytopenia.
abstract: Previously, we described a group of patients with hemocytopenia who did not conform to diagnostic criteria of known hematological and nonhematological diseases. Most patients responded well to adrenocortical hormone and/or high-dose intravenous immunoglobulin treatment, indicating that cytopenia might be mediated by autoantibodies. Autoantibodies were detected on the membrane of various bone marrow (BM) hemopoietic cells by bone marrow mononuclear-cell-Coombs test or flow cytometric analysis. Thus, the hemocytopenia was termed "Immunorelated Pancytopenia" (IRP) to distinguish it from other pancytopenias. Autoantigens in IRP were investigated by membrane protein extraction from BM hemopoietic cells and BM supernatant from IRP patients. Autoantibody IgG was detected in the BM supernatant of 75% of patients (15/20), which was significantly higher than that in aplastic anemia, myelodysplastic syndrome, or autoimmune hemolytic anemia patients (0%) and normal healthy controls (0%) (P < 0.01). Autoantigens had approximate molecular weights of 25, 30, 47.5, 60, 65, 70, and 80 kDa, some of which were further identified by mass fingerprinting. This study identified that a G-protein-coupled receptor 156 variant and chain P, a crystal structure of the cytoplasmic domain of human erythrocyte band-3 protein, were autoantigens in IRP. Further studies are needed to confirm the antigenicity of these autoantigens.
authors: Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan Street, Heping District, Tianjin 300052, China.
authors: Hui
authors: H
authors: Liu
authors: Rong
authors: R
authors: Fu
authors: Yihao
authors: Y
authors: Wang
authors: Hong
authors: H
authors: Liu
authors: Lijuan
authors: L
authors: Li
authors: Honglei
authors: H
authors: Wang
authors: Jin
authors: J
authors: Chen
authors: Hong
authors: H
authors: Yu
authors: Zonghong
authors: Z
authors: Shao
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000276:immunology / Q000378:metabolism*; D001324:Autoantigens / Q000276:immunology / Q000378:metabolism*; D001854:Bone Marrow Cells / Q000276:immunology*; D002478:Cells, Cultured; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology / Q000378:metabolism*; D056747:Immunomodulation; D008297:Male; D010198:Pancytopenia / Q000175:diagnosis* / Q000276:immunology; D040901:Proteomics; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D007074:Immunoglobulin G