title: Comparison of immune manifestations between refractory cytopenia of childhood and aplastic anemia in children: A single-center retrospective study.
abstract: This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis.
authors.affiliation: Department of Pediatrics, Medical School, Peking University, The Affiliated People's Hospital of Peking University, Peking, China.
authors.forename: Jun
authors.initials: J
authors.lastname: Wu
authors.affiliation: Department of Pediatrics, Medical School, Peking University, The Affiliated People's Hospital of Peking University, Peking, China. Electronic address: chengyifei182@sina.com.
authors.forename: Yifei
authors.initials: Y
authors.lastname: Cheng
authors.affiliation: Department of Pediatrics, Medical School, Peking University, The Affiliated People's Hospital of Peking University, Peking, China.
authors.forename: Leping
authors.initials: L
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D000361:Agammaglobulinemia / Q000453:epidemiology; D000741:Anemia, Aplastic / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology / Q000276:immunology / Q000473:pathology; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003176:Complement C3 / Q000172:deficiency; D003181:Complement C4 / Q000172:deficiency; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D016694:Gene Rearrangement, delta-Chain T-Cell Antigen Receptor; D006801:Humans; D006942:Hypergammaglobulinemia / Q000453:epidemiology; D007136:Immunoglobulins / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D018655:Lymphocyte Count; D016131:Lymphocyte Subsets / Q000473:pathology; D008297:Male; D009190:Myelodysplastic Syndromes / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology / Q000276:immunology / Q000473:pathology; D012189:Retrospective Studies; D013997:Time Factors
chemical_list: D000961:Antilymphocyte Serum; D001323:Autoantibodies; D003176:Complement C3; D003181:Complement C4; D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D016572:Cyclosporine
keywords: Aplastic anemia; Children; Immune manifestation; Refractory cytopenia