Akbarzadehpasha Amirhossein, Lotfi Zahra, Omidvar Razieh, Goodarzi Azadeh, Hosseini Saeid, Sadeghzadeh Bazargan Afsaneh, Kamyab Hesari Kambiz
Rajaie Cardiovascular Medical and Research Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
These authors contributed equally in writing this article.
Caspian J Intern Med. 2024 Oct 27;16(1):178-184. doi: 10.22088/cjim.16.1.178. eCollection 2025 Winter.
Mastocytosis is a systemic disease involving the clonal expansion of mast cells in multiple organs. Given that immune system overreaction and excessive histamine release are among the most prominent events in mastocytosis, the incidence of complications caused by immune reactions is expected to increase across various organs. While systemic manifestations of mastocytosis have been reported frequently, cardiac complications are less often discussed. These Cardiac complications can be early indicators of the disease but such uncommon features may lead to delays in diagnosis.The significance of mast cells and histamine release in the cardiovascular system is acknowledged in prior studies.
This study presents a case of recurrent prosthetic mitral valve malfunction in a 52-year-old patient with a history of cutaneous mastocytosis, who underwent mitral valve replacement three times over ten years. Despite being on appropriate anticoagulation therapy (INR: 2.5-3.5), the patient experienced recurrent prosthetic valve thrombosis. This is, to our knowledge, the first report of prosthetic mitral valve thrombosis in a patient with mastocytosis.
Interestingly, cardiac complications may be the first presentation of systemic mastocytosis, diagnosed long after the initial symptoms. The majority of such cases had no visible cutaneous manifestations (table 1). Regarding our case report, we recommend our colleagues to closely monitor and remain vigilant for possible cardiac symptoms of mastocytosis patients with prosthetic cardiac valves.
肥大细胞增多症是一种全身性疾病,涉及多个器官中肥大细胞的克隆性增殖。鉴于免疫系统过度反应和组胺过度释放是肥大细胞增多症中最突出的事件,预计免疫反应引起的并发症在各个器官中的发生率都会增加。虽然肥大细胞增多症的全身表现经常被报道,但心脏并发症较少被讨论。这些心脏并发症可能是该疾病的早期指标,但这种不常见的特征可能导致诊断延迟。先前的研究已经认识到肥大细胞和组胺释放在心血管系统中的重要性。
本研究报告了一例52岁有皮肤肥大细胞增多症病史的患者反复出现人工二尖瓣功能障碍的病例,该患者在十年内接受了三次二尖瓣置换术。尽管接受了适当的抗凝治疗(国际标准化比值:2.5 - 3.5),患者仍反复出现人工瓣膜血栓形成。据我们所知,这是肥大细胞增多症患者人工二尖瓣血栓形成的首例报告。
有趣的是,心脏并发症可能是全身性肥大细胞增多症的首发表现,在初始症状出现很久之后才被诊断出来。大多数此类病例没有明显的皮肤表现(表1)。关于我们的病例报告,我们建议同事们密切监测并警惕有心脏人工瓣膜的肥大细胞增多症患者可能出现的心脏症状。