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维奈托克联合低甲基化剂治疗髓样肉瘤并干细胞移植:罕见病例报告。

The myeloid sarcoma treated by Venetoclax with hypomethylating agent followed by stem cell transplantation: rare case report.

机构信息

Personalized Medicine Centre, Almazov National Medical Research Centre, 2 Akkuratova Str., Saint Petersburg, Russian Federation, 197341.

Almazov National Medical Research Centre, 2 Akkuratova Str., Saint Petersburg, Russian Federation, 197341.

出版信息

BMC Womens Health. 2021 May 1;21(1):184. doi: 10.1186/s12905-021-01328-y.

Abstract

BACKGROUND

Myeloid sarcoma (MS) is a very rare condition, develops both in patients with other hematological neoplasms, and as isolated tumor. MS of the gynecologic tract is extremely rare. An available literature data about diagnosis and management of MS is summarized in the article. The role of chemotherapy, radiation therapy, surgery and bone marrow transplantation in the treatment is discussed. Polychemotherapy and allogeneic bone marrow transplantation were suggested to be the optimal treatment strategy of MS of the gynecological tract. The use of new targeted agents results in promising clinical data.

CASE PRESENTATION

We are presenting a rare clinical case of a MS of the uterine cervix with concomitant bone marrow involvement and describe all the peculiarities of the clinical course, diagnosis, and treatment. The patient received chemotherapy followed by allogeneic bone marrow transplantation. The pre-transplant therapy allowed us to perform allogeneic bone marrow transplantation with the deepest response possible: complete PET-negative and MRD-negative remission of the disease.

CONCLUSIONS

MS remains a subject of discussion regarding its diagnostic and therapeutic aspects. The use of novel targeting agents can be perspective option for patient with extramedullary disease.

摘要

背景

骨髓肉瘤(MS)是一种非常罕见的疾病,既可以在患有其他血液系统肿瘤的患者中发展,也可以作为孤立性肿瘤出现。妇科生殖道 MS 极为罕见。本文总结了 MS 的诊断和治疗方面的可用文献数据。讨论了化疗、放疗、手术和骨髓移植在治疗中的作用。多化疗和异基因骨髓移植被认为是妇科生殖道 MS 的最佳治疗策略。新型靶向药物的使用带来了有前景的临床数据。

病例介绍

我们报告了一例罕见的宫颈 MS 合并骨髓受累的病例,并描述了其所有临床病程、诊断和治疗的特点。该患者接受了化疗,随后进行了异基因骨髓移植。移植前的治疗使我们能够进行异基因骨髓移植,获得尽可能深的反应:疾病完全 PET 阴性和 MRD 阴性缓解。

结论

MS 在其诊断和治疗方面仍是一个讨论的主题。新型靶向药物的使用可能是治疗骨髓外疾病的有前途的选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb46/8088676/7c6664dfa517/12905_2021_1328_Fig1_HTML.jpg

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