Sada Eriko, Shiratsuchi Motoaki, Ohno Hirofumi, Nagasawa Eriko, Honda Emi, Takayanagi Ryoichi, Abe Yasunobu
Department of Medicine and Bioregulatory Science, Kyushu University, Kyushu, Japan.
Rinsho Ketsueki. 2011 May;52(5):293-8.
We report here a very severe case of thymoma-related aplastic anemia that developed after thymectomy. The patient was a 50-year-old man diagnosed with myasthenia gravis. Chest CT showed thymoma measuring 7.5 cm in diameter, and extended thymectomy was performed. Irradiation to the anterior mediastinum was added postoperatively. Thirteen months after surgery, hemogram showed severe neutropenia: leukocyte count 0.32×10(9)/l with 11% neutrophils; Hb 10.7 g/dl; and platelet count 100×10(9)/l. Although cyclosporine (CSP, 5 mg/kg/day) was administered, dose reduction was necessary because of renal damage. Cytopenia deteriorated to a leukocyte count of 0.71×10(9)/l with 21% neutrophils; Hb 5.9 g/dl; and platelet count 24×10(9)/l. However, addition of antithymocyte globulin (ATG, 15 mg/kg) led to hematopoietic recovery of all three lineages within one month. He is clinically stable with no transfusion requirement after 22 months with CSP maintenance therapy. Although thymoma-related aplastic anemia has been reported to have an extremely poor prognosis, high efficacy of CSP has been reported recently. In our case, ATG in combination with CSP was efficient. Adequate immunosuppressive therapy seems to be important for the clinical management of these patients.
我们在此报告一例胸腺切除术后发生的极为严重的胸腺瘤相关再生障碍性贫血病例。患者为一名50岁男性，诊断为重症肌无力。胸部CT显示直径7.5厘米的胸腺瘤，遂行扩大胸腺切除术。术后加行前纵隔放疗。术后13个月，血常规显示严重中性粒细胞减少：白细胞计数0.32×10⁹/L，中性粒细胞占11%；血红蛋白10.7 g/dl；血小板计数100×10⁹/L。尽管给予环孢素（CSP，5毫克/千克/天）治疗，但因肾损害需减少剂量。血细胞减少恶化，白细胞计数降至0.71×10⁹/L，中性粒细胞占21%；血红蛋白5.9 g/dl；血小板计数24×10⁹/L。然而，加用抗胸腺细胞球蛋白（ATG，15毫克/千克）后，所有三个血细胞系在1个月内实现造血恢复。在接受CSP维持治疗22个月后，他临床稳定，无需输血。尽管胸腺瘤相关再生障碍性贫血的预后据报道极差，但最近有报道称CSP疗效显著。在我们的病例中，ATG联合CSP有效。充分的免疫抑制治疗对这些患者的临床管理似乎很重要。
