Georgiou Georgios K, Gizas Ioannis, Katopodis Konstantinos P, Katsios Christos S
Department of Surgery, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece.
Department of Nephrology, University Hospital of Ioannina, Stavros Niarchos Avenue, 45500, Ioannina, Greece.
Surg Today. 2015 Oct;45(10):1317-20. doi: 10.1007/s00595-014-1073-3. Epub 2014 Nov 6.
Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder, which may be idiopathic or secondary to a variety of diseases. However, there are very few reports of TTP in the context of pancreatic neoplasms. We report a case of relapsing TTP after initial treatment with plasmapheresis, corticosteroids, and rituximab, in a 59-year-old woman. During diagnostic work-up, a pancreatic lesion 35 × 25 mm in size was discovered incidentally and splenopancreatectomy was performed. The pathological diagnosis was benign glucagonoma. The hematological symptoms resolved completely after the procedure and 3 years later, the patient is well with no sign of recurrence of TTP or glucagonoma. To our knowledge, this represents the first documented case of a non-secreting benign pancreatic neuroendocrine tumor (glucagonoma) associated with TTP that is refractory to standard treatment.
血栓性血小板减少性紫癜（TTP）是一种罕见的血液系统疾病，可能是特发性的，也可能继发于多种疾病。然而，关于胰腺肿瘤合并TTP的报道非常少。我们报告一例59岁女性患者，在接受血浆置换、皮质类固醇和利妥昔单抗初始治疗后复发的TTP。在诊断检查过程中，偶然发现一个大小为35×25mm的胰腺病变，并进行了脾胰切除术。病理诊断为良性胰高血糖素瘤。术后血液学症状完全缓解，3年后，患者情况良好，无TTP或胰高血糖素瘤复发迹象。据我们所知，这是首例记录在案的与TTP相关的非分泌性良性胰腺神经内分泌肿瘤（胰高血糖素瘤），且对标准治疗无效。
