{"title":"一例罕见的Waldenström巨球蛋白血症表现为双侧血性胸腔积液和全血细胞减少症。","authors":"Yue Qing Chen, Hua Wang, Qiong Feng","doi":"10.12659/AJCR.948815","DOIUrl":null,"url":null,"abstract":"<p><p>BACKGROUND Waldenström macroglobulinemia (WM) is an uncommon, indolent mature B-cell lymphoma with various clinical manifestations. Bilateral bloody pleural effusion is rare, while chylothorax is even more infrequent. Furthermore, pancytopenia holds significant clinical relevance in WM, highlighting clinicians' need to exercise substantial vigilance. CASE REPORT We report the case of a 71-year-old man who was admitted for chest tightness, dyspnea, and fatigue. Initial laboratory tests revealed pancytopenia, and a computed tomography (CT) scan showed bilateral pleural effusion. The diagnostic workup included a lymph node biopsy that confirmed CD20+ B-cell lymphoma. Bone marrow molecular studies identified somatic mutations in MYD88 (L252P, c.755T>C) and CXCR4 (R322Sfs*16, c. 966-982del). The bone marrow biopsy indicated lymphoplasmacytic lymphoma. A serum immunofixation test revealed monoclonal bands of IgM and kappa. Based on the patient's symptoms and examination results, a thorough analysis led to the final diagnosis of Waldenström macroglobulinemia. The patient underwent therapeutic thoracentesis, followed by 4 cycles of rituximab (375 mg/m² on Day 1) and bendamustine (70 mg/m² on Days 2-3). Post-treatment evaluation revealed hematologic recovery and complete resolution of pleural effusions on thoracic ultrasound. CONCLUSIONS This case report elaborates on Waldenström macroglobulinemia's diagnosis and treatment process to enhance clinicians' awareness and ability to manage the complications associated with this rare disease.</p>","PeriodicalId":39064,"journal":{"name":"American Journal of Case Reports","volume":"26 ","pages":"e948815"},"PeriodicalIF":0.7000,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A Rare Case of Waldenström Macroglobulinemia Presenting as Bilateral Bloody Pleural Effusion and Pancytopenia.\",\"authors\":\"Yue Qing Chen, Hua Wang, Qiong Feng\",\"doi\":\"10.12659/AJCR.948815\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>BACKGROUND Waldenström macroglobulinemia (WM) is an uncommon, indolent mature B-cell lymphoma with various clinical manifestations. Bilateral bloody pleural effusion is rare, while chylothorax is even more infrequent. Furthermore, pancytopenia holds significant clinical relevance in WM, highlighting clinicians' need to exercise substantial vigilance. CASE REPORT We report the case of a 71-year-old man who was admitted for chest tightness, dyspnea, and fatigue. Initial laboratory tests revealed pancytopenia, and a computed tomography (CT) scan showed bilateral pleural effusion. The diagnostic workup included a lymph node biopsy that confirmed CD20+ B-cell lymphoma. Bone marrow molecular studies identified somatic mutations in MYD88 (L252P, c.755T>C) and CXCR4 (R322Sfs*16, c. 966-982del). The bone marrow biopsy indicated lymphoplasmacytic lymphoma. A serum immunofixation test revealed monoclonal bands of IgM and kappa. Based on the patient's symptoms and examination results, a thorough analysis led to the final diagnosis of Waldenström macroglobulinemia. The patient underwent therapeutic thoracentesis, followed by 4 cycles of rituximab (375 mg/m² on Day 1) and bendamustine (70 mg/m² on Days 2-3). Post-treatment evaluation revealed hematologic recovery and complete resolution of pleural effusions on thoracic ultrasound. CONCLUSIONS This case report elaborates on Waldenström macroglobulinemia's diagnosis and treatment process to enhance clinicians' awareness and ability to manage the complications associated with this rare disease.</p>\",\"PeriodicalId\":39064,\"journal\":{\"name\":\"American Journal of Case Reports\",\"volume\":\"26 \",\"pages\":\"e948815\"},\"PeriodicalIF\":0.7000,\"publicationDate\":\"2025-10-03\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"American Journal of Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.12659/AJCR.948815\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.12659/AJCR.948815","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
摘要
背景Waldenström巨球蛋白血症(WM)是一种罕见的、惰性的成熟b细胞淋巴瘤,具有多种临床表现。双侧血性胸腔积液是罕见的,而乳糜胸则更为罕见。此外,全细胞减少症在WM中具有重要的临床相关性,强调临床医生需要保持高度警惕。病例报告我们报告一例71岁男性因胸闷、呼吸困难和疲劳入院。最初的实验室检查显示全血细胞减少,计算机断层扫描显示双侧胸腔积液。诊断检查包括淋巴结活检,证实CD20+ b细胞淋巴瘤。骨髓分子研究发现MYD88 (L252P, C . 755t >C)和CXCR4 (R322Sfs*16, C . 966-982del)的体细胞突变。骨髓活检显示淋巴浆细胞性淋巴瘤。血清免疫固定试验显示IgM和kappa单克隆条带。根据患者的症状和检查结果,经过彻底的分析,最终诊断为Waldenström巨球蛋白血症。患者接受治疗性胸腔穿刺,随后给予4个周期的利妥昔单抗(375 mg/m²,第1天)和苯达莫司汀(70 mg/m²,第2-3天)。治疗后胸部超声检查显示血液学恢复,胸膜积液完全消除。结论本病例报告阐述了Waldenström巨球蛋白血症的诊断和治疗过程,以提高临床医生对这种罕见疾病相关并发症的认识和管理能力。
A Rare Case of Waldenström Macroglobulinemia Presenting as Bilateral Bloody Pleural Effusion and Pancytopenia.
BACKGROUND Waldenström macroglobulinemia (WM) is an uncommon, indolent mature B-cell lymphoma with various clinical manifestations. Bilateral bloody pleural effusion is rare, while chylothorax is even more infrequent. Furthermore, pancytopenia holds significant clinical relevance in WM, highlighting clinicians' need to exercise substantial vigilance. CASE REPORT We report the case of a 71-year-old man who was admitted for chest tightness, dyspnea, and fatigue. Initial laboratory tests revealed pancytopenia, and a computed tomography (CT) scan showed bilateral pleural effusion. The diagnostic workup included a lymph node biopsy that confirmed CD20+ B-cell lymphoma. Bone marrow molecular studies identified somatic mutations in MYD88 (L252P, c.755T>C) and CXCR4 (R322Sfs*16, c. 966-982del). The bone marrow biopsy indicated lymphoplasmacytic lymphoma. A serum immunofixation test revealed monoclonal bands of IgM and kappa. Based on the patient's symptoms and examination results, a thorough analysis led to the final diagnosis of Waldenström macroglobulinemia. The patient underwent therapeutic thoracentesis, followed by 4 cycles of rituximab (375 mg/m² on Day 1) and bendamustine (70 mg/m² on Days 2-3). Post-treatment evaluation revealed hematologic recovery and complete resolution of pleural effusions on thoracic ultrasound. CONCLUSIONS This case report elaborates on Waldenström macroglobulinemia's diagnosis and treatment process to enhance clinicians' awareness and ability to manage the complications associated with this rare disease.
期刊介绍:
American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.