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一例罕见的恶性胸腺瘤相关移植物抗宿主病病例。

An unusual case of malignant thymoma associated graft-versus-host disease.

作者信息

Gishen Faye Sara, Tookman Adrian J

机构信息

Marie Curie Cancer Care, Edenhall Hospice, Hampstead, 11 Lyndhurst Gardens, London NW3 5NS, UK.

出版信息

BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.05.2009.1866. Epub 2009 Aug 19.

Abstract

A woman in her early 50s presented with recurrent severe chest infections. Investigations revealed a low white cell count and a diagnosis of autoimmune neutropenia was made. Subsequently, an infiltrating thymic tumour (mitoses only) in the absence of myasthenia gravis was found. She underwent radical surgery. When neutropenic, she complained of painful, swollen joints and soft tissues. She was started on steroids and immunosuppressants and her pain settled. The following year, she had local malignant recurrence confirmed on imaging. She declined chemotherapy or targeted somatostatin and opted for alternative therapies. She developed a microcytic anaemia and commenced erythropoietin. This coincided with the development of a painful expanded rib lesion, hypercalcaemia, and ascites. She remained unwell with periodical flares in disease affecting many different organs and continued to mount a significant immunological response to her thymic tumour, manifesting as biopsy proven graft-versus-host disease involving joints, skin and lungs. This has been a complex clinical case involving multiple specialities, including haematology, oncology, immunology, endocrinology and palliative medicine.

摘要

一名50岁出头的女性因反复出现严重的胸部感染前来就诊。检查发现白细胞计数偏低,诊断为自身免疫性中性粒细胞减少症。随后,在无重症肌无力的情况下发现了一例浸润性胸腺肿瘤(仅见有丝分裂)。她接受了根治性手术。中性粒细胞减少时,她诉说关节和软组织疼痛、肿胀。她开始使用类固醇和免疫抑制剂,疼痛得到缓解。次年,影像学检查证实肿瘤局部复发。她拒绝化疗或靶向生长抑素治疗,选择了替代疗法。她出现了小细胞贫血,并开始使用促红细胞生成素。与此同时,出现了疼痛性肋骨膨胀性病变、高钙血症和腹水。她病情持续不佳,疾病时有发作,累及多个不同器官,并继续对胸腺肿瘤产生显著的免疫反应,表现为经活检证实的移植物抗宿主病,累及关节、皮肤和肺部。这是一个复杂的临床病例,涉及多个专科,包括血液学、肿瘤学、免疫学、内分泌学和姑息医学。

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