Malkan Umit Yavuz, Albayrak Murat, Ozturk Hacer Berna, Reis Aras Merih, Saglam Bugra, Dogan Kutsal
University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Department of Hematology, Ankara, Turkey.
University of Health Sciences, Diskapi Yildirim Beyazit Training and Research Hospital, Department of Pathology, Ankara, Turkey.
Case Rep Oncol. 2020 Nov 26;13(3):1368-1372. doi: 10.1159/000510745. eCollection 2020 Sep-Dec.
Microangiopathic hemolytic anemia (MAHA) can be observed as a paraneoplastic syndrome (PS) in certain tumors. MAHA-related signet ring cell carcinoma (SRCC) of an unknown origin is very infrequent. Herein we present a SRCC case presented with refractory acquired thrombotic thrombocytopenic purpura (TTP). A 35-year-old man applied to the emergency service with fatigue and headache. His laboratory tests resulted as white blood cell 9,020/µL, hemoglobin 3.5 g/dL, platelet 18,000/µL. Schistocytes, micro-spherocytes, and thrombocytopenia were observed in his blood smear. MAHA was present and he was considered as having TTP. Plasma exchange treatment was initiated; however, he was refractory to this treatment. Thorax and abdomen computerized tomography revealed thickening of minor curvature in stomach corpus with hepatogastric and paraceliac lymphadenopathy. Bone marrow (BM) investigation by our clinic resulted as the metastasis of adenocarcinoma. Ulceration and necrosis were observed by gastric endoscopy procedure. Biopsy was taken during endoscopic intervention, which resulted as SRCC. MAHA may be seen as a PS in some tumors, especially gastric cancers. Tumor-related MAHA is generally accompanied by BM metastases. As a result, BM investigation may be used as the main diagnostic method to find the underlying cancer. The clinical course of cases with tumor-related MAHA is usually poor, and these cases are usually refractory to plasma exchange treatment. In conclusion, physicians should suspect a malignancy and BM involvement when faced with a case of refractory TTP.
微血管病性溶血性贫血（MAHA）在某些肿瘤中可表现为副肿瘤综合征（PS）。起源不明的MAHA相关印戒细胞癌（SRCC）非常罕见。在此，我们报告一例表现为难治性获得性血栓性血小板减少性紫癜（TTP）的SRCC病例。一名35岁男性因疲劳和头痛前往急诊。他的实验室检查结果为白细胞9,020/µL，血红蛋白3.5 g/dL，血小板18,000/µL。在他的血涂片中观察到裂红细胞、微球形红细胞和血小板减少。存在MAHA，他被认为患有TTP。开始进行血浆置换治疗；然而，他对此治疗无效。胸部和腹部计算机断层扫描显示胃体小弯增厚，伴有肝胃和腹腔旁淋巴结肿大。我们诊所进行的骨髓（BM）检查结果为腺癌转移。胃镜检查发现溃疡和坏死。在内镜干预期间进行活检，结果为SRCC。MAHA在某些肿瘤，尤其是胃癌中可能表现为PS。肿瘤相关的MAHA通常伴有BM转移。因此，BM检查可作为发现潜在癌症的主要诊断方法。肿瘤相关MAHA病例的临床病程通常较差，并且这些病例通常对血浆置换治疗无效。总之，当面对难治性TTP病例时，医生应怀疑存在恶性肿瘤和BM受累。
