Tokunaga Masahiro, Yamada Masahide, Kondo Atsushi, Mishima Mai, Yoshikawa Shinichi, Inoue Shinya, Morita Takako, Tominaga Nobuhiko
Department of Hematology, Suita Municipal Hospital.
Department of Central Laboratory, Suita Municipal Hospital.
Rinsho Ketsueki. 2018;59(3):310-314. doi: 10.11406/rinketsu.59.310.
Adult-onset autoimmune neutropenia (AIN) is rarely self-limiting, unlike infant-onset AIN. Although several therapeutic agents have been reported, including corticosteroids, more effective treatment options may exist. Here, we describe neutrophil recovery by eltrombopag in a 52-year-old male AIN patient with immune thrombocytopenia (ITP) who was referred to our hospital with severe neutropenia. Within a year of referral, he developed moderate thrombocytopenia. He was diagnosed with AIN and concurrent ITP, based on the detection of antineutrophil antibodies and bone marrow aspiration, respectively. Further platelet count reduction and the appearance of purpura prompted an initial treatment of 0.5 mg/kg prednisolone. Thrombocytopenia remission was prompt but transient, with platelet counts rapidly declining before initiating prednisolone tapering. Similarly, absolute neutrophil counts (ANCs), after a shorter recovery period, returned to the baseline level below 2×10/l. Further platelet reduction was prevented by eltrombopag administration. Intriguingly, the ANCs recovered following platelet recovery and remained above 5×10/l for > three months despite prednisolone dosage tapering. To our knowledge, this is the first report describing the effectiveness of eltrombopag in AIN.
与婴幼儿期发病的自身免疫性中性粒细胞减少症(AIN)不同,成人期发病的AIN很少会自行缓解。尽管已有多种治疗药物被报道,包括皮质类固醇,但可能存在更有效的治疗选择。在此,我们描述了艾曲泊帕使一名52岁患有免疫性血小板减少症(ITP)的男性AIN患者中性粒细胞恢复的情况,该患者因严重中性粒细胞减少症转诊至我院。在转诊后的一年内,他出现了中度血小板减少症。分别基于抗中性粒细胞抗体的检测和骨髓穿刺,他被诊断为AIN并发ITP。进一步的血小板计数降低和紫癜的出现促使给予0.5mg/kg泼尼松龙进行初始治疗。血小板减少症缓解迅速但短暂,在开始逐渐减少泼尼松龙剂量之前血小板计数迅速下降。同样,绝对中性粒细胞计数(ANC)在较短的恢复期后,又回到了低于2×10⁹/L的基线水平。给予艾曲泊帕可防止血小板进一步减少。有趣的是,在血小板恢复后ANC也恢复了,并且尽管泼尼松龙剂量逐渐减少,但ANC在超过三个月的时间里一直保持在5×10⁹/L以上。据我们所知,这是第一份描述艾曲泊帕对AIN有效性的报告。