Wilms Tumor and Paraneoplastic Syndromes: Unusual Presentation With Acquired von Willebrand Disease, Polycythemia and Hyperviscosity-A Comprehensive Case Report and Literature Reviews.

IF 0.9 4区 医学 Q4 HEMATOLOGY
Marina Parisi Dutra, Lilian Maria Cristofani, Maria Teresa Assis de Almeida, Vicente Odone Filho, Roberto Augusto Plaza Teixeira
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引用次数: 0

Abstract

A 10-month-old child presenting with renal tumor with polycythemia, acquired von Willebrand disease, and elevated serum hyaluronic acid, leading to a diagnosis of Wilms tumor. Laboratory profile showed elevated levels of hemoglobin and hematocrit, prolonged aPTT, and high levels of hyaluronic acid and erythropoietin, which normalized after chemotherapy and tumor resection. She had a complete response and has remained in remission. Paraneoplastic syndromes are rare manifestations of Wilms tumor (WT). The simultaneous occurrence of distinct types, as demonstrated in this report (polycythemia, hyperviscosity, and coagulopathy), has not been previously reported. This unique presentation poses a challenge for both diagnostic and clinical management.

Wilms肿瘤和副肿瘤综合征:获得性血管性血友病、红细胞增多症和高粘血症的不寻常表现——一个综合病例报告和文献综述。
一个10个月大的婴儿,表现为肾肿瘤伴红细胞增多症,获得性血管性血友病,血清透明质酸升高,导致肾母细胞瘤的诊断。实验室资料显示血红蛋白和红细胞压积升高,aPTT延长,透明质酸和促红细胞生成素水平升高,化疗和肿瘤切除后恢复正常。她有了完全的反应,并一直处于缓解期。副肿瘤综合征是肾母细胞瘤(WT)的罕见表现。同时发生的不同类型,如本报告所示(红细胞增多症、高黏度和凝血功能障碍),以前未见报道。这种独特的表现对诊断和临床管理都提出了挑战。
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来源期刊
CiteScore
1.90
自引率
8.30%
发文量
415
审稿时长
2.5 months
期刊介绍: ​Journal of Pediatric Hematology/Oncology (JPHO) reports on major advances in the diagnosis and treatment of cancer and blood diseases in children. The journal publishes original research, commentaries, historical insights, and clinical and laboratory observations.
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