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华氏巨球蛋白血症伪装成伴有腹膜癌病、腹水及CA-125升高的卵巢癌。

Waldenström's macroglobulinemia masquerading as ovarian cancer with peritoneal carcinomatosis, ascites, and elevated CA-125.

作者信息

Eulitt Patrick, Fabian Denise, Kelly Crystal, Hemminger Jessica, William Basem M

机构信息

Ohio State University, Wexner Medical Center, 460 West 10th Avenue, Columbus, OH 43210, USA.

Ohio State University, Wexner Medical Center, 460 West 10th Avenue, Columbus, OH 43210, USA.

出版信息

Hematol Oncol Stem Cell Ther. 2019 Mar;12(1):54-59. doi: 10.1016/j.hemonc.2017.02.004. Epub 2017 Mar 31.

Abstract

Waldenström's macroglobulinemia is a rare hematology malignancy which often presents with "B symptoms," anemia, and thrombocytopenia. A 46-year-old woman presented with 2 months of abdominal distension accompanied by an unintentional 20-lb weight loss. Her abdominal CT scan demonstrated diffuse carcinomatosis with bilateral ovarian lesions and screening labs revealed a markedly elevated CA-125, suggesting a diagnosis of ovarian cancer. Upon admission for workup, patient was found to have a significant protein gap, later attributed to a markedly elevated IgM. Omental and bone marrow biopsy confirmed the diagnosis of Waldenström's macroglobulinemia, with elevation in CA-125 thought to be secondary to peritoneal irritation. This patient has since been successfully treated with six cycles of bendamusine and rituximab with no evidence of disease on staging scans and normalization of both CA-125 and IgM. To our knowledge, this is the first documented case of Waldenström's macroglobulinemia presenting with symptoms classically associated with ovarian cancer and demonstrates the importance of maintaining a broad differential when evaluating patients with abdominal carcinomatosis.

摘要

华氏巨球蛋白血症是一种罕见的血液系统恶性肿瘤,常表现为“B症状”、贫血和血小板减少。一名46岁女性出现2个月的腹胀,并伴有体重意外减轻20磅。她的腹部CT扫描显示弥漫性癌病伴双侧卵巢病变,筛查实验室检查显示CA-125显著升高,提示卵巢癌诊断。入院检查时,发现患者存在明显的蛋白差距,后来发现是由于IgM显著升高所致。网膜和骨髓活检确诊为华氏巨球蛋白血症,CA-125升高被认为是继发于腹膜刺激。该患者此后接受了六个周期的苯达莫司汀和利妥昔单抗治疗,分期扫描未发现疾病迹象,CA-125和IgM均恢复正常。据我们所知,这是首例有文献记载的表现出与卵巢癌经典相关症状的华氏巨球蛋白血症病例,证明了在评估有腹部癌病的患者时保持广泛鉴别诊断的重要性。

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