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一名多发性骨髓瘤患者获得性因子X缺乏症的病例报告。

A Case Report of Acquired Factor X Deficiency in a Patient With Multiple Myeloma.

作者信息

Sabobeh Taher, Brugioni Emily K, Masoud Amgad, Madhusudhana Sheshadri, Mateescu Valerica

机构信息

Internal Medicine, University of Missouri Kansas City, Kansas City, USA.

Hematology and Oncology, University of Missouri Kansas City, Kansas City, USA.

出版信息

Cureus. 2021 Feb 11;13(2):e13293. doi: 10.7759/cureus.13293.

DOI:10.7759/cureus.13293
PMID:33728224
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7949742/
Abstract

Multiple myeloma is a plasma cell neoplasm characterized by clonal proliferation of immunoglobulin producing terminally differentiated B cells. Classically patients are described to present with bone pain, hypercalcemia, anemia, and/or renal impairment. A less described clinical manifestation related to the myeloma is acquired coagulation abnormalities including paraprotein interfering with the coagulation cascade or exhibiting specific antibody activity. Factor X deficiency is reported in patients with secondary amyloidosis. We describe a patient who presented with bleeding tendency and an abnormal prothrombin and activated partial thromboplastin times (PT/PTT) due to factor X deficiency. A thorough workup revealed the diagnosis of multiple myeloma with the presence of monoclonal lambda light chain restricted plasma cells with qualifying end-organ damage without evidence of amyloidosis. Prior to the ultimate diagnosis, the patient succumbed to septic shock and acute respiratory distress syndrome due to infection.

摘要

多发性骨髓瘤是一种浆细胞肿瘤,其特征为产生免疫球蛋白的终末分化B细胞的克隆性增殖。传统上,患者表现为骨痛、高钙血症、贫血和/或肾功能损害。与骨髓瘤相关的一种较少描述的临床表现是获得性凝血异常,包括副蛋白干扰凝血级联反应或表现出特定抗体活性。继发性淀粉样变性患者中报告有因子X缺乏。我们描述了一名因因子X缺乏而出现出血倾向以及凝血酶原和活化部分凝血活酶时间(PT/PTT)异常的患者。全面检查显示诊断为多发性骨髓瘤,存在单克隆λ轻链受限的浆细胞,并伴有符合条件的终末器官损害,且无淀粉样变性证据。在最终诊断之前,患者因感染死于感染性休克和急性呼吸窘迫综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/7949742/f6200c76d9fc/cureus-0013-00000013293-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/7949742/03312e5b2d5c/cureus-0013-00000013293-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/7949742/f6200c76d9fc/cureus-0013-00000013293-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/7949742/03312e5b2d5c/cureus-0013-00000013293-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/7949742/f6200c76d9fc/cureus-0013-00000013293-i02.jpg

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引用本文的文献

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Acquired FX Deficiency in Multiple Myeloma without Concomitant Amyloidosis: A Rare Case Report.多发性骨髓瘤伴发非淀粉样变性的获得性因子X缺乏症:一例罕见病例报告
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本文引用的文献

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A Rare Case of Early Onset Multiple Myeloma in a 20-Year-Old Female With Factor X Inhibitor.一名20岁患有X因子抑制剂的女性早发性多发性骨髓瘤罕见病例。
J Hematol. 2018 May;7(2):69-71. doi: 10.14740/jh380w. Epub 2018 May 10.
2
Acquired factor X deficiency in a patient with multiple myeloma: a rare case highlighting the significance of comprehensive evaluation and the need for antimyeloma therapy for bleeding diathesis.一名多发性骨髓瘤患者获得性因子X缺乏症:一个罕见病例凸显全面评估的重要性以及针对出血素质进行抗骨髓瘤治疗的必要性。
BMJ Case Rep. 2019 Sep 16;12(9):e230249. doi: 10.1136/bcr-2019-230249.
3
Acquired Factor X Deficiency in Patients With Primary Light Chain Amyloidosis.
原发性轻链淀粉样变性患者获得性因子X缺乏症
J Investig Med High Impact Case Rep. 2019 Jan-Dec;7:2324709619832332. doi: 10.1177/2324709619832332.
4
Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease.轻链(AL)淀粉样变性中获得性因子X缺乏症罕见,且与晚期疾病相关。
Hematol Oncol Stem Cell Ther. 2019 Mar;12(1):10-14. doi: 10.1016/j.hemonc.2018.05.002. Epub 2018 Jun 28.
5
Risk factors for infections in newly diagnosed Multiple Myeloma patients: A Danish retrospective nationwide cohort study.初诊多发性骨髓瘤患者感染的危险因素:丹麦全国回顾性队列研究。
Eur J Haematol. 2019 Feb;102(2):182-190. doi: 10.1111/ejh.13190. Epub 2018 Nov 28.
6
Factor X Deficiency Caused by Nonsecretory Myeloma Successfully Corrected with Bortezomib: A Case Report and Review of the Literature.硼替佐米成功纠正非分泌性骨髓瘤所致的X因子缺乏:1例报告及文献复习
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7
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Asian Pac J Cancer Prev. 2018 Jan 27;19(1):127-130. doi: 10.22034/APJCP.2018.19.1.127.
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Am Fam Physician. 2017 Mar 15;95(6):373-383.
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Experience of a new high-purity factor X concentrate in subjects with hereditary factor X deficiency undergoing surgery.新型高纯度X因子浓缩物用于遗传性X因子缺乏症患者手术的经验。
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