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具有三重威胁的胸腺瘤:纯红细胞再生障碍、自身免疫性溶血性贫血和T细胞大颗粒淋巴细胞白血病。

Thymoma With Triple Threat: Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia, and T-Cell Large Granular Lymphocytic Leukemia.

作者信息

Seibert Tara, Loehrer Patrick J, O'Brien Andrew R W

机构信息

Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Division of Hematology/Oncology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

出版信息

J Hematol. 2022 Dec;11(6):223-232. doi: 10.14740/jh1061. Epub 2022 Dec 30.

Abstract

Thymomas are a rare neoplasm of the anterior mediastinum and often associated with paraneoplastic syndromes. Though myasthenia gravis is the most common and well-known, the list of reported paraneoplastic syndromes occurring with thymoma is extensive and ever-growing. Paraneoplastic syndromes can involve nearly every organ system, including hematologic abnormalities affecting any or all cell lines. This can present challenges to the clinician in terms of diagnosis, prognostic impact, and management. We present the case of a previously healthy 41-year-old female who was diagnosed with thymoma and three rare hematologic paraneoplastic syndromes: pure red cell aplasia (PRCA), autoimmune hemolytic anemia (AIHA), and T-cell large granular lymphocytic leukemia (T-LGLL). To the best of our knowledge, there have been only four other reported cases of PRCA and AIHA in a single patient with thymoma, all of which were treated with thymectomy. Upfront surgical resection was not possible in the present case and thus the patient was alternatively treated with corticosteroids and octreotide, which proved successful in resolving the anemia. The authors present this case to share these findings of an alternative treatment strategy for thymoma-associated PRCA and AIHA and to highlight the importance of careful monitoring with routine blood work for these complex patients.

摘要

胸腺瘤是前纵隔的一种罕见肿瘤,常与副肿瘤综合征相关。虽然重症肌无力是最常见且广为人知的,但与胸腺瘤相关的已报道副肿瘤综合征的列表很广泛且不断增加。副肿瘤综合征可累及几乎每个器官系统,包括影响任何或所有细胞系的血液学异常。这在诊断、预后影响和管理方面会给临床医生带来挑战。我们报告一例先前健康的41岁女性病例,她被诊断为胸腺瘤并伴有三种罕见的血液学副肿瘤综合征:纯红细胞再生障碍性贫血(PRCA)、自身免疫性溶血性贫血(AIHA)和T细胞大颗粒淋巴细胞白血病(T-LGLL)。据我们所知,仅有另外四例报告称一名胸腺瘤患者同时患有PRCA和AIHA,所有这些病例均接受了胸腺切除术治疗。在本病例中无法进行早期手术切除,因此该患者改用皮质类固醇和奥曲肽治疗,结果证明这在解决贫血方面是成功的。作者展示此病例是为了分享胸腺瘤相关PRCA和AIHA的替代治疗策略的这些发现,并强调对这些复杂患者进行常规血液检查仔细监测的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3514/9822658/d2033cfcdb6d/jh-11-223-g001.jpg

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