METHODS: A 76-year-old female was referred to our hospital due to Coombs-negative hemolytic anemia, which was causing fatigue and dyspnea on exertion, accompanied by schistocytosis. A bone marrow examination demonstrated bone marrow carcinomatosis, and the tumor cells were morphologically suspected to be signet-ring cell carcinoma cells. As we failed to find the primary tumor site before the patient died, she was diagnosed with CR-TMA due to bone marrow carcinomatosis of unknown primary origin. Thrombotic thrombocytopenic purpura (TTP) was rapidly ruled out based on her PLASMIC score. In addition, immunohistochemical staining of a clot section of the bone marrow and tumor marker data were useful for narrowing down the likely primary tumor site.
CONCLUSIONS: Although CR-TMA is an extremely rare phenomenon, clinicians who suspect CR-TMA should quickly rule out TTP and decide whether to provide appropriate chemotherapy or plan for palliative care.
方法:一名76岁女性因Coombs阴性溶血性贫血转诊至我院,导致劳累时疲劳和呼吸困难,伴有血吸虫病。骨髓检查显示骨髓癌,肿瘤细胞在形态上怀疑为印戒细胞癌细胞。由于我们未能在患者死亡前找到原发肿瘤部位,由于原发不明的骨髓癌病,她被诊断为CR-TMA.根据她的PLASMIC评分迅速排除血栓性血小板减少性紫癜(TTP)。此外,骨髓凝块切片的免疫组织化学染色和肿瘤标志物数据有助于缩小可能的原发肿瘤部位。
结论:尽管CR-TMA是一种极为罕见的现象,怀疑CR-TMA的临床医生应迅速排除TTP,并决定是否提供适当的化疗或姑息治疗计划.