Yagasaki Hideaki, Inukai Takeshi, Uno Kanako, Akahane Kosi, Nemoto Atsushi, Takahashi Kazuya, Sato Hiroki, Goi Kumiko, Mouri Naruaki, Takano Kunio, Katoh Ryohei, Sugita Kanji, Nakazawa Shinpei
Department of Pediatrics, University of Yamanashi School of Medicine.
Rinsho Ketsueki. 2003 May;44(5):328-33.
A 14-year-old girl was admitted because of general fatigue and cervical lymphadenopathy. She showed bilateral struma (IInd degree) and enlargement of her left cervical lymph nodes. Laboratory data revealed neutropenia (219/microliter) and thrombocytopenia (Plt 5.1 x 10(4)/microliter) with mild anemia (Hb 11.1 g/dl), and the bone marrow aspirate and biopsy specimens showed hypocellularity. In addition, auto-antibodies against thyroid peroxidase (TPO) and thyroglobulin (TG) were highly elevated. Computed tomography of the neck showed a nodule in the left thyroid lobe with marked lymphadenopathy, and fine needle aspiration biopsy demonstrated papillary thyroid carcinoma with Hashimoto's thyroiditis and metastasis to the lymph nodes. One month after left thyroid lobectomy and cervical lymphadenectomy, the patient's condition progressed to very severe aplastic anemia, and she received immunosuppressive therapy consisting of cyclosporin A and anti-thymocyte globulin. Hematologically, partial and complete responses were obtained three and six months later, respectively. Of interest, anti-TPO and TG antibody titers remarkably decreased after immunosuppressive therapy. The patient had HLA-DR 2(DRB 11501) and DR 8(DRB 10802). The former is frequently found in patients with cyclosporin A-dependent aplastic anemia, and the latter is frequently found in Asian patients with Hashimoto's thyroiditis, suggesting an underlying autoimmune background for the simultaneous outbreak of aplastic anemia and Hashimoto's thyroiditis complicated by thyroid carcinoma.
一名14岁女孩因全身乏力和颈部淋巴结肿大入院。她有双侧甲状腺肿大(II度)以及左侧颈部淋巴结肿大。实验室检查数据显示中性粒细胞减少(219/微升)、血小板减少(血小板计数5.1×10⁴/微升)伴轻度贫血(血红蛋白11.1克/分升),骨髓穿刺和活检标本显示细胞减少。此外,抗甲状腺过氧化物酶(TPO)和抗甲状腺球蛋白(TG)自身抗体高度升高。颈部计算机断层扫描显示左甲状腺叶有一个结节并伴有明显的淋巴结病,细针穿刺活检显示为乳头状甲状腺癌合并桥本甲状腺炎及淋巴结转移。在左甲状腺叶切除术和颈部淋巴结清扫术后一个月,患者病情进展为非常严重的再生障碍性贫血,她接受了由环孢素A和抗胸腺细胞球蛋白组成的免疫抑制治疗。血液学方面,分别在三个月和六个月后获得了部分缓解和完全缓解。有趣的是,免疫抑制治疗后抗TPO和TG抗体滴度显著下降。该患者有HLA-DR 2(DRB 11501)和DR 8(DRB 10802)。前者常见于依赖环孢素A的再生障碍性贫血患者,后者常见于亚洲桥本甲状腺炎患者,提示再生障碍性贫血与桥本甲状腺炎合并甲状腺癌同时发作存在潜在的自身免疫背景。