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儿童白血病相关性阴茎异常勃起(LAPC):临床结局与管理策略综述

Leukaemia-associated priapism in children (LAPC): reviewing clinical outcomes and management strategies.

作者信息

Shah Abhijit, Surag K R, Choudhary Anupam, Viswanath Kasi, Krishnakanth Avb, Krishna Chaitanya, Hegde Padmaraj, Gayathri S, Swathi P M

机构信息

Department of Urology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.

Department of Clinical Hematology, Cellular therapy and Bone marrow transplant, Amrita Institute of Medica Sciences, Kochi, Kerala, India.

出版信息

Ecancermedicalscience. 2025 Feb 27;19:1860. doi: 10.3332/ecancer.2025.1860. eCollection 2025.

Abstract

BACKGROUND

Priapism is a prolonged penile erection for more than 4 hours unrelated to sexual stimulation. Rarely, it is the first clinical sign of an underlying haematological malignancy. A similar presentation is noted in childhood leukaemias. Although rare, it is known to occur and, if not managed early, can have poor long-term outcomes in terms of erectile function and psychosexual growth. We present a scoping review of leukaemia-associated priapism in children (LAPC).

METHODOLOGY

We researched literature using PubMed, Google Scholar, Embase, Scopus and Cochrane databases from January 1990 to 2024. Applicable search limiters were applied, and grey literature was excluded.

RESULTS

A total of 31 articles were finally included in the review, from which 51 cases of LAPC were isolated and studied. The average age was 11.5 years, with chronic myeloid leukaemia (CML) being the most common malignancy (68.9%), and more than 71% of cases of CML with priapism were detected in the chronic phase. Twenty cases (39.2%) were managed with corporal lavage and sympathomimetic injections at the initial onset, with the rest managed with cytoreductive measures initially. Follow-up data revealed the death of three children, whereas, of those that survived, fourteen had preserved erectile functions after a variable period of time.

CONCLUSION

Priapism in children warrants a thorough physical examination focusing on organomegaly and a complete hemogram. Initial management should be two-pronged with a priapism-directed corporal-lavage approach and cytoreductive measures for better long-term outcomes.

摘要

背景

阴茎异常勃起是指阴茎持续勃起超过4小时且与性刺激无关。极少数情况下,它是潜在血液系统恶性肿瘤的首个临床症状。儿童白血病也有类似表现。虽然罕见,但已知会发生,如果不及早处理,在勃起功能和性心理发育方面可能会有不良的长期后果。我们对儿童白血病相关阴茎异常勃起(LAPC)进行了一项范围综述。

方法

我们使用PubMed、谷歌学术、Embase、Scopus和Cochrane数据库检索了1990年1月至2024年的文献。应用了适用的检索限制器,并排除了灰色文献。

结果

该综述最终纳入了31篇文章,从中分离并研究了51例LAPC病例。平均年龄为11.5岁,慢性髓性白血病(CML)是最常见的恶性肿瘤(68.9%),超过71%的CML伴阴茎异常勃起病例在慢性期被检测到。20例(39.2%)在发病初期采用阴茎灌洗和拟交感神经药物注射治疗,其余病例最初采用细胞减灭措施治疗。随访数据显示有3名儿童死亡,而在存活的儿童中,有14名在一段时间后保留了勃起功能。

结论

儿童阴茎异常勃起需要进行全面的体格检查,重点关注器官肿大情况和全血细胞计数。初始治疗应双管齐下,采用针对阴茎异常勃起的阴茎灌洗方法和细胞减灭措施,以获得更好的长期疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/324f/12010180/b8b2f879c533/can-19-1860fig1.jpg

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