A Signet Ring Cell Carcinoma Presented as Refractory Acquired Thrombotic Thrombocytopenic Purpura

Abstract

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.

Cancer‐related thrombotic microangiopathy and disseminated intravascular coagulation in a patient with bone marrow carcinomatosis of unknown primary origin: A case report

“…Concerning diagnosis, CR‐TMA is generally accompanied by multiple bone or bone marrow metastases. Hence, bone marrow investigations are useful as a primary diagnostic method for identifying the underlying occult carcinoma 6,12 . In the present case, the presence of an underlying causative tumor was strongly suspected because of the patient's high carcinoembryonic antigen and neuron‐specific enolase levels, and a diagnosis of epithelial carcinoma was made after a bone marrow examination.…”

“…Hence, bone marrow investigations are useful as a primary diagnostic method for identifying the underlying occult carcinoma. 6,12 In the present case, the presence of an underlying causative tumor was strongly suspected because of the patient's high carcinoembryonic antigen and neuron-specific enolase levels, and a diagnosis of epithelial carcinoma was made after a bone marrow examination. Clusters of tumor cells were observed in a smear as well as in a clot section, leading to a diagnosis of bone marrow carcinomatosis and related TMA.…”