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骨髓增生异常综合征继 Good 综合征:与胸腺瘤相关的独特表现。

Myelodysplasia followed by Good's Syndrome: a unique manifestation associated with thymoma.

机构信息

Division of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

出版信息

Kaohsiung J Med Sci. 2012 Apr;28(4):236-40. doi: 10.1016/j.kjms.2011.10.012. Epub 2012 Feb 11.

Abstract

Good's syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good's syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good's syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good's syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.

摘要

Good 综合征,又称伴免疫缺陷的胸腺瘤,较为罕见。免疫缺陷可能先于、与胸腺瘤同时或之后出现。除重症肌无力和 Good 综合征外,胸腺瘤相关的副肿瘤综合征还可表现为血液系统疾病,如纯红细胞再生障碍、再生障碍性贫血、粒细胞缺乏症、溶血性贫血、恶性贫血和阵发性睡眠性血红蛋白尿。骨髓增生异常综合征是一组克隆性造血干细胞疾病,其特征为血细胞减少、一条或多条谱系发育不良、无效造血和急性白血病前体。骨髓增生异常综合征的一种发病机制是自身抗体直接排斥造血细胞,但这种情况在胸腺瘤中很少见。在此,我们报告了一例伴独特副肿瘤综合征的胸腺瘤患者,该患者先于 Good 综合征发生骨髓增生异常。早期和准确诊断骨髓增生异常综合征对疾病管理很重要,尤其是在骨髓增生异常综合征可能源于自身免疫的患者中。对于反复感染的胸腺瘤患者,有必要进行全面的免疫研究以排除 Good 综合征的可能性,并在合适的患者中预防性静脉注射免疫球蛋白输注。

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