Franco Andre Silva, Polho Gabriel Berlingieri, Luppino Assad Ana Paula, Miossi Renata, Sampaio-Barros Percival Degrava
Division of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
Instituto do Câncer do Estado de São Paulo, Faculty of Medicine, University of Sao Paulo, Sao Paulo, Brazil.
J Scleroderma Relat Disord. 2023 Oct;8(3):NP9-NP13. doi: 10.1177/23971983231171248. Epub 2023 Apr 28.
The overlapping of systemic sclerosis with hematologic malignancy has been described previously in the literature. This case report presents a patient with systemic sclerosis and multiple myeloma who had severe digital ischaemia that culminated in the amputation of several fingers.
A 65-year-old White female patient was diagnosed with limited systemic sclerosis in 2002, smouldering multiple myeloma IgG/kappa in 2017 and liver cirrhosis in 2018 due to autoimmune hepatitis. In 2021, she was admitted to the emergency room with dry ischaemia of all fingers and toes despite optimized therapy, associated with visual blurring. The diagnostic hypothesis was hyperviscosity syndrome associated with multiple myeloma reactivation. The patient underwent chemotherapy and despite initial laboratory improvement, 19 digits required amputation.
Although the association between systemic sclerosis and multiple myeloma is rare, it should be remembered in cases of significant worsening of Raynaud's phenomenon. Causes unrelated to systemic sclerosis should also be considered in the presence of severe exacerbations in patients with other comorbidities.
系统性硬化症与血液系统恶性肿瘤的重叠此前已有文献报道。本病例报告介绍了一名患有系统性硬化症和多发性骨髓瘤的患者,该患者出现严重的手指缺血,最终导致多个手指被截肢。
一名65岁的白人女性患者于2002年被诊断为局限性系统性硬化症,2017年被诊断为冒烟型多发性骨髓瘤IgG/κ,2018年因自身免疫性肝炎被诊断为肝硬化。2021年,尽管接受了优化治疗,她仍因所有手指和脚趾干性缺血并伴有视力模糊而被送入急诊室。诊断假设为与多发性骨髓瘤复发相关的高黏滞综合征。患者接受了化疗,尽管最初实验室检查有所改善,但仍有19个手指需要截肢。
尽管系统性硬化症与多发性骨髓瘤之间的关联罕见,但在雷诺现象显著恶化的病例中应予以考虑。在患有其他合并症的患者出现严重病情加重时,也应考虑与系统性硬化症无关的病因。