An unusual presentation of EATL type 1: Emergency surgery due to life-threatening gastrointestinal bleeding
A 76-years-old male admitted to emergency service with sudden and severe gastrointestinal bleeding and hypovolemic shock. There were massive hematochezia and hematemesis. There is no history of celiac disease in his/family history. Systolic arterial blood pressure was 60 mmHg, diastolic was unmeasurable. The patient was monitored and resuscitation were started immediately. Crystalloids and erythrocyte suspensions were given intravenously. After the initial resuscitation hemodynamic stability was provided and then the patient transferred to Intensive Care Unit (ICU). The hemoglobin value increased from 4 mg/100 mL to 8 mg/100 mL. Emergency upper and lower gastrointestinal endoscopy revealed no lesion or active bleeding. There was blood clots in the colon and stomach. A short time later, second severe bleeding was observed in ICU. Despite the resuscitation and transfusions, the patient re-entered the hypovolemic shock. We decided to emergency laparatomy in doubt.\nIn exploration there was an adhesion between the transverse mesocolon and the jejunum just after the ligament of Trietz. We divided the adhesion. There was a perforated lesion that had been bleeding, on antimesenteric surface of jejunum (). Perforated area was attached to adjecent intestine so that there was no blood in peritoneal cavity. We resected the lesion with margins of a few centimeters of healthy jejunum in both sides. End-to-end anastomosis was performed.\nIn histopathological examination, macroscopically, there were traces of bleeding and almost complete necrosis. Microscopically, intestinal mucosa showed extensive ulceration and necrosis. Extensive necrosis made the diagnosis very hard. But the following findings led us to the diagnosis of EATL type 1. The muscularis propria had abundant atypical lymphoid infiltrate in diffuse pattern (a). Neoplastic lymphocytes were medium to large sized cells with irregular nuclear contours and hyperchromatic nuclei. Some large cells with vesicular nuclei had prominent nucleoli (c). The jejunal mucosa adjacent to the tumor showed effacement of normal villous architecture (villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis, pyloric metaplasia) (a and b). Immunohistochemically, atypical lymphoid cells positive for CD3 (b) and CD30 (d). They were immunonegative for CD56, CD4, CD8, CD20, ALK1. The background population was composed of reactive B and T lymphocytes. The intraepithelial lymphocytes in the adjacent mucosa were positive for CD3 and negative for CD4 and CD8.\nThe patient was discharged after surgical healing and referred to hematology clinic in eighth day postoperatively. The patient received combined chemotherapy regimen composed of bleomycin, doxorubicin, cyclophosphamide, vincristine, and prednisone. Since then he has been followed up by 18F-fluorodeoxyglucose positron emission tomography. The patient is alive in twenty-fourth month after diagnosis, there were no findings suggesting recurrence or metastasis. He continues to strict gluten-free diet.