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以小肠梗阻为表现的侵袭性髓外多发性骨髓瘤

Aggressive Extramedullary Multiple Myeloma Presenting as Small Bowel Obstruction.

作者信息

Wurtz Paul J, McGovern Kevin, Shah Jamie, Stoll Kristin E, Moore Devin

机构信息

Department of Internal Medicine, Brooke Army Medical Center, Fort Sam Houston, TX 78234, USA.

Department of General Surgery, Brooke Army Medical Center, Fort Sam Houston, TX 78234, USA.

出版信息

J Hematol. 2025 Apr;14(2):86-93. doi: 10.14740/jh2031. Epub 2025 Feb 25.

DOI:10.14740/jh2031
PMID:40336918
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12056742/
Abstract

extramedullary multiple myeloma (EMM) is a rare subset of multiple myeloma (MM) defined by the presence of clonal plasma cells (PC) outside of the bone marrow. It is associated with refractory disease and adverse outcomes. Even in EMM, plasmacytomas within the duodenum, jejunum, and ileum are uncommon, with fewer than 70 cases reported in the literature. Here, we present a particularly aggressive case of EMM resulting in a small bowel obstruction secondary to an intraluminal plasmacytoma while on myeloma-directed therapy. The patient underwent surgical resection with anastomosis and was transitioned to more definitive cytotoxic chemotherapy followed by autologous stem cell rescue. This case highlights challenges in the management of EMM over standard MM and argues that dedicated clinical trials for patients with aggressive EMM are warranted to further understand the unique pathophysiology and improve overall survival.

摘要

髓外多发性骨髓瘤(EMM)是多发性骨髓瘤(MM)的一种罕见亚型,其定义为骨髓外存在克隆性浆细胞(PC)。它与难治性疾病和不良预后相关。即使在EMM中,十二指肠、空肠和回肠内的浆细胞瘤也不常见,文献报道的病例少于70例。在此,我们报告一例特别侵袭性的EMM病例,该病例在接受骨髓瘤定向治疗时,因腔内浆细胞瘤导致小肠梗阻。患者接受了吻合术的手术切除,并转而接受更确切的细胞毒性化疗,随后进行自体干细胞救援。该病例突出了EMM在管理上相较于标准MM的挑战,并表明有必要针对侵袭性EMM患者开展专门的临床试验,以进一步了解其独特的病理生理学并提高总体生存率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/f86afa2fb46e/jh-14-02-086-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/2b1c3248abfd/jh-14-02-086-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/39871d557008/jh-14-02-086-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/57d9dac095a7/jh-14-02-086-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/f86afa2fb46e/jh-14-02-086-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/2b1c3248abfd/jh-14-02-086-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/39871d557008/jh-14-02-086-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/57d9dac095a7/jh-14-02-086-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7490/12056742/f86afa2fb46e/jh-14-02-086-g004.jpg

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本文引用的文献

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Natural history, predictors of development of extramedullary disease, and treatment outcomes for patients with extramedullary multiple myeloma.髓外多发性骨髓瘤患者的自然病史、髓外疾病发展的预测因素和治疗结果。
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