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卵巢睾丸间质细胞瘤导致的高雄激素血症伴红细胞增多症。

Hyperandrogenism due to ovarian Leydig cell tumour presenting with polycythaemia.

机构信息

Department of Clinical Chemistry and Haematology, Meander Medical Centre, Amersfoort, The Netherlands

Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands.

出版信息

BMJ Case Rep. 2022 Jul 15;15(7):e249651. doi: 10.1136/bcr-2022-249651.

DOI:10.1136/bcr-2022-249651
PMID:35840164
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9295641/
Abstract

A postmenopausal woman in her 60s was referred due to an elevated haemoglobin value found during her annual check-up. On physical examination, characteristic features of hyperandrogenism were observed which were not earlier mentioned. Laboratory investigations revealed polycythaemia accompanied by a normal erythropoietin and a negative analysis for JAK2-V617F mutation. A disproportionally and markedly elevated testosterone in combination with normal levels of adrenal androgens raised the suspicion of an ovarian source. CT scan showed nodular hyperdense lesions in both ovaries. A bilateral oophorectomy was performed and histological evaluation unfolded a Leydig cell ovarian tumour. Testosterone levels and haematological parameters normalised after surgery. Polycythaemia secondary to hyperandrogenism in postmenopausal women is an extremely rare condition and patients should be carefully analysed for the presence of androgen-secreting neoplasms. Diagnosis of the underlying pathology requires careful history, physical examination and comprehensive investigation. Treatment for this condition is surgery and resolves polycythaemia.

摘要

一位 60 多岁的绝经后女性因年度体检发现血红蛋白值升高而被转介。体格检查观察到了高雄激素血症的特征性表现,而这些表现之前并未提及。实验室检查显示红细胞增多症伴正常的促红细胞生成素和 JAK2-V617F 突变阴性分析。睾酮不成比例地显著升高,同时肾上腺雄激素水平正常,这增加了卵巢来源的怀疑。CT 扫描显示双侧卵巢结节状高密度病变。行双侧卵巢切除术,组织学评估显示为睾丸间质细胞瘤。手术后,睾酮水平和血液学参数恢复正常。绝经后女性因高雄激素血症引起的红细胞增多症是一种极其罕见的情况,应仔细分析是否存在雄激素分泌性肿瘤。明确潜在病理需要仔细的病史、体格检查和全面的检查。这种情况下的治疗方法是手术,可解决红细胞增多症。

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本文引用的文献

1
Practical Review of Ovarian Sex Cord-Stromal Tumors.卵巢性索间质肿瘤实用综述
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Erythrocytosis Following Testosterone Therapy.睾酮治疗后的红细胞增多症。
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Occult leydig cell tumour and androgen-receptor positive breast cancer in a woman with severe hyperandrogenism.女性严重高雄激素血症患者中的隐匿性睾丸间质细胞瘤和雄激素受体阳性乳腺癌。
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No metabolic impact of surgical normalization of hyperandrogenism in postmenopausal women with ovarian androgen-secreting tumours.绝经后卵巢雄激素分泌肿瘤妇女手术纠正高雄激素血症对代谢无影响。
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Clinical syndromes associated with ovarian neoplasms: a comprehensive review.与卵巢肿瘤相关的临床综合征:全面综述。
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