Comparison of immune manifestations between refractory cytopenia of childhood and aplastic anemia in children: A single-center retrospective study. Issue 12 (December 2015)

• Record Type: Journal Article
• Authors: Wu, Jun; Cheng, Yifei; Zhang, Leping
• Is Part Of: Leukemia research. Volume 39:Issue 12(2015:Dec.)
• Page Start: 1347
• Page End: 1352
• Publication Date: 2015-12
• Subjects: AA aplastic anemia -- AIM autoimmune manifestation -- ATG anti-thymocyte globulin -- CR complete remission -- MDS myelodysplastic syndromes -- PR partial remission -- RCC refractory cytopenia of childhood -- TCR T-cell receptor; Immune manifestation -- Children -- Refractory cytopenia -- Aplastic anemia; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2015.09.012
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: We evaluated children with refractory cytopenia (RCC) or aplastic anemia (AA). Immune abnormalities were common in both RCC and AA. There was considerable overlap between the two groups. Thus, RCC and AA may partly share the same pathogenesis 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, χ 2 = 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, χ 2 = 0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis.