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一名塞浦路斯β-地中海贫血妇女罕见的肾上腺髓外造血病例。

A rare case of adrenal extramedullary haematopoiesis in a Cypriot woman with β-thalassaemia.

机构信息

European University Cyprus, Nicosia, Cyprus.

Makarios Thalassemia Hospital Nicosia, Cyprus.

出版信息

Ann R Coll Surg Engl. 2024 Apr;106(4):329-337. doi: 10.1308/rcsann.2021.0298. Epub 2022 Jan 4.

Abstract

We report a rare case of adrenal extramedullary haematopoiesis (EMH) in a thalassaemia patient in Cyprus. A 40-year-old woman with β-thalassaemia presented with a 2-day history of non-specific right-sided abdominal pain on routine follow-up for her thalassaemia treatment. Her laboratory tests were not dissimilar to her routine results and no palpable mass was detected. Computed tomography findings revealed a 5.8×4.2×4.6cm solid lesion in the right adrenal gland. Surgical excision was advised for this symptomatic large tumour with the possibility of malignancy in a young patient, and a laparoscopic adrenalectomy was performed. Postoperative follow-up was uneventful. A review of the literature in PubMed and MEDLINE revealed 14 case reports worldwide with adrenal EMH secondary to β-thalassaemia. EMH tumours in patients with thalassaemia have been reported incidentally, which stresses the importance of considering this in the list of differentials of adrenal incidentalomas in this patient population.

摘要

我们报告了一例塞浦路斯地中海贫血患者罕见的肾上腺髓外造血(EMH)病例。一名 40 岁的女性β-地中海贫血患者,在常规接受地中海贫血治疗的随访中出现了 2 天的非特异性右侧腹痛。她的实验室检查与常规结果无明显差异,也未触及肿块。计算机断层扫描结果显示右侧肾上腺有一个 5.8×4.2×4.6cm 的实性病变。鉴于年轻患者有恶性肿瘤的可能,且该肿瘤为症状性大肿瘤,建议进行手术切除,随后进行了腹腔镜肾上腺切除术。术后随访无异常。在 PubMed 和 MEDLINE 上查阅文献后发现,全球范围内有 14 例继发于β-地中海贫血的肾上腺 EMH 病例报告。在偶然情况下报道了地中海贫血患者的 EMH 肿瘤,这强调了在该患者人群的肾上腺意外瘤鉴别诊断列表中考虑这一情况的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/87ee/10981986/ec0f80fa5d09/rcsann.2021.0298.01.jpg

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