Adachi Masaaki
Hematology Division, JCHO Sapporo Hokushin Hospital.
Rinsho Ketsueki. 2016;57(12):2512-2516. doi: 10.11406/rinketsu.57.2512.
Simultaneous onset of autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) is rare and any possible association between these two disorders remains obscure. A 46-year-old previously healthy woman was diagnosed as having AIHA based on severe anemia, positive direct and indirect Coomb's tests, decreased serum haptoglobin, elevated serum LDH, and indirect bilirubin-dominant hyperbilirubinemia. Oral steroid administration (1 mg/kg) and subsequent half-pulse steroid therapy ameliorated the AIHA, but the anemia was unexpectedly prolonged with the low peripheral blood reticulocyte count further decreasing to 0.11%. Bone marrow aspiration revealed a marked decrease in erythroblasts with an M/E ratio of 69.5. Anti-parvovirus B19 IgM antibody and serum B19 viral DNA (10 copy/ml) were detected but no other distinct abnormalities which might have caused acquired PRCA were detected. Therefore, she was considered likely to have idiopathic AIHA and acquired PRCA simultaneously. AIHA-mediated erythroblastosis probably raised the parvovirus B19 DNA level to an extraordinary degree and thereby led to severe aplastic crisis, subsequently causing prolonged anemia. Parvovirus B19 infection should be considered in AIHA patients showing unexpectedly low reticulocyte counts.
自身免疫性溶血性贫血(AIHA)与纯红细胞再生障碍性贫血(PRCA)同时发病较为罕见,这两种疾病之间的任何可能关联仍不明确。一名46岁既往健康的女性,基于严重贫血、直接和间接抗人球蛋白试验阳性、血清触珠蛋白降低、血清乳酸脱氢酶升高以及以间接胆红素为主的高胆红素血症,被诊断为AIHA。口服类固醇(1mg/kg)及随后的半脉冲类固醇治疗使AIHA病情改善,但贫血意外延长,外周血网织红细胞计数进一步降至0.11%。骨髓穿刺显示成红细胞显著减少,M/E比值为69.5。检测到抗细小病毒B19 IgM抗体和血清B19病毒DNA(10拷贝/ml),但未检测到其他可能导致获得性PRCA的明显异常。因此,她被认为可能同时患有特发性AIHA和获得性PRCA。AIHA介导的成红细胞增多可能使细小病毒B19 DNA水平异常升高,从而导致严重的再生障碍危象,随后引起贫血延长。对于网织红细胞计数意外降低的AIHA患者,应考虑细小病毒B19感染。