上海交通大学学报（医学版）

上海交通大学学报（医学版） ›› 2024, Vol. 44 ›› Issue (4): 537-542.doi: 10.3969/j.issn.1674-8115.2024.04.016

• 病例报告 • 上一篇    

多发性骨髓瘤伴心脏淀粉样变性并继发性肾上腺皮质功能减退症1例

周凌云(), 陈海飞()   

  1. 广东省深圳市罗湖区人民医院血液内科，深圳 518000
  • 收稿日期:2023-07-14 接受日期:2024-02-06 出版日期:2024-04-28 发布日期:2024-04-28
  • 通讯作者: 陈海飞 E-mail:flyhighzhou@163.com;chhf1224@163.com
  • 作者简介:周凌云（1987—），女，主治医师，硕士；电子信箱：flyhighzhou@163.com。

Multiple myeloma with cardiac amyloidosis and secondary adrenal cortical dysfunction: a case report

ZHOU Lingyun(), CHEN Haifei()   

  1. Department of Hematology, Shenzhen Luohu People's Hospital, Guangdong Province, Shenzhen 518000, China
  • Received:2023-07-14 Accepted:2024-02-06 Online:2024-04-28 Published:2024-04-28
  • Contact: CHEN Haifei E-mail:flyhighzhou@163.com;chhf1224@163.com

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摘要：

多发性骨髓瘤是一种浆细胞恶性增殖性疾病，部分患者可合并系统性淀粉样变性，心脏淀粉样变性是该类患者常见的死亡原因。继发性肾上腺功能不全是由下丘脑和/或垂体功能障碍所致，而多发性骨髓瘤患者合并继发性肾上腺皮质功能减退症的病例在国内鲜见报道。患者，55岁，男性，2018年6月5日因“反复胸闷、乏力7个月，晕厥1 h”就诊于深圳市罗湖区人民医院急诊科，后转入血液内科，经骨髓穿刺等检查明确诊断为多发性骨髓瘤（λ轻链型）伴系统性淀粉样变性。化学治疗（化疗）第1疗程方案为硼替佐米、环磷酰胺和地塞米松，疗程结束后患者肺部出现细菌与真菌混合感染，经治疗后好转。第2疗程开始方案调整为硼替佐米和地塞米松，第4疗程后患者多发性骨髓瘤即达到完全缓解；第5疗程后患者再次发生严重肺部混合感染，治疗后好转，但后续出现顽固性低血压，皮质醇和促肾上腺皮质激素（adrenocorticotropic hormone，ACTH）水平下降，诊断为继发性肾上腺皮质功能减退症，予以氢化可的松替代治疗。患者接受9个化疗疗程后，以伊沙佐米维持治疗，多发性骨髓瘤评估为严格意义的完全缓解状态，心脏淀粉样变性疗效评估为非常好的部分缓解；继发性肾上腺皮质功能减退症以氢化可的松维持治疗，皮质醇水平在正常范围内。

关键词: 多发性骨髓瘤, 系统性淀粉样变性, 心脏淀粉样变性, 继发性肾上腺皮质功能减退症

Abstract:

Multiple myeloma is a malignant proliferative disease of plasma cells, and some patients may develop systemic amyloidosis. Cardiac amyloidosis is a common cause of death in these patients. Secondary adrenal insufficiency is caused by dysfunction of the hypothalamus and/or the pituitary gland, and multiple myeloma cases combined with secondary adrenal cortical dysfunction have been rarely reported in China. The patient, 55-year-old, male, was admitted to the Emergency Department of Shenzhen Luohu People's Hospital on June 5, 2018 due to "repeated chest tightness and fatigue for 7 months, and fainting for 1 hour". Later, he was transferred to Department of Hematology and was diagnosed as having multiple myeloma (λ light chain type) with systemic amyloidosis through bone marrow puncture and other examinations. The first course regimen of chemotherapy was bortezomib, cyclophosphamide, and dexamethasone. After the course, the patient was infected with a mixture of bacteria and fungi in the lung and had improvement after treatment. Then the regimen was adjusted to bortezomib and dexamethasone from the second course. After the fourth course, the patient achieved complete remission of multiple myeloma. After the fifth course, the patient experienced severe pulmonary-mixed infection again, which was improved after treatment. Thereafter, the patient presented with refractory hypotension, and decreased levels of cortisol and adrenocorticotropic hormone (ACTH), which was diagnosed as secondary adrenal cortical dysfunction. Hydrocortisone replacement therapy was administered. After 9 courses of chemotherapy, the patient received maintenance treatment with ixazomib. Multiple myeloma was evaluated as a stringent complete response. Cardiac amyloidosis was evaluated as a very good partial response, and secondary adrenal cortical dysfunction was treated with hydrocortisone maintenance therapy and with the cortisol level in the normal range.

Key words: multiple myeloma, systemic amyloidosis, cardiac amyloidosis, secondary adrenal cortical dysfunction

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