Anorectal and gastric peripheral T-cell lymphoma, unspecified in a non-AIDS patient
A 29-year-old man was referred to our hospital with complaints of hematochezia and tenesmus for 2 months. He underwent colonofiberscopic examination without biopsy by a primary clinician. The colonofiberscopic examination showed a huge multiple fungating submucosal mass at the anorectal region (). On admission, the patient complained hematochezia, tenesmus and abdominal discomfort, but there was no specific findings noted on the abdominal physical examination. The vital signs revealed a blood pressure of 100/70 mmHg, a pulse rate of 68/min, a respiratory rate of 22/min and his temperature was 36.5℃. The laboratory test showed a white blood cell count of 9,110/µL (84.1% polymorphonuclear cells and 9.2% lymphocytes), a hemoglobin level of 12.3 g/dL, a hematocrit of 36.8%, a platelet count of 412,000/µL, an erythrocyte sedimentation rate of 10 mm/hour and a C-reactive protein level of 0.03 mg/dL (normal range: 0-0.5). The partial thromboplastin time and prothrombin time were within normal ranges. The results of liver function tests were as follows protein 8.7 g/dL, albumin 4.5 g/dL, bilirubin 1.6 mg/dL, aspartate transaminase 26 IU/L, alanine transaminase 53 IU/L, alkaline phosphatase 51 IU/L, cholesterol 138 mg/dL and lactic dehydrogenase (LDH) 514 IU/L. The renal function tests were normal. The serum electrolytes showed sodium 138 mM/dL, potassium 4.9 Mm/L, chloride 100 mM/L, calcium 9.2 mg/dL and phosphate 4.8 mg/dL. Urinalysis was negative for occult blood and there was a trace of protein. No antibodies against syphilis, hepatitis B, hepatitis C or human immunodeficiency virus were detected. Under the clinical impression of anal and rectal submucosal tumor, the patient underwent excisional biopsy. The histologic findings of the specimen showed dense infiltration of angulated lymphoid cells into the lamina propria together with perivascular infiltrations (). On immunohistochemistry, these lymphoid cells were positive for CD3 (), lymphocyte common antigen, Ki-67 and CD45RO, but they were negative for CD20 and CD56. These morphologic features and the immunophenotype profiles were compatible with PTCLu. For further evaluation, he underwent gastrofiberscopy and bone marrow biopsy. Gastrofiberscopic examination showed mottled or patched hyperemic mucosa and round-shaped, flat elevated lesions on the gastric antrum (). Biopsy of the gastric lesion gave the same diagnosis, but there was no lymphoma that involved the bone marrow. The chest CT scan was normal; the abdominal-pelvic CT scan showed wall thickening from the lower rectum to the rectosigmid junction, and there were multiple lymphadenopathies in the perirectal area, sigmoid mesocolon and paraaortic area, and enlarged nodes were noted along the hepatoduodenal ligament.\nFinally, the patient was diagnosed as suffering with rectal and gastric PTCLu. The Ann Arbor stage was IVA and the age-adjusted international prognostic index was 3. He was in the high risk group. He was treated with six cycles of combination chemotherapy including cyclophosphamide, adriamycin, vincristine, etoposide and prednisone (CHOEP). After two cycles of chemotherapy, the initially observed rectal wall thickening disappeared on the follow-up abdominal-pelvic CT scan; we observed markedly decreased size of the lymph nodes of the paraaortic and portocarval space. On follow-up gastrofiberscopic biopsy, a few lymphoid cells had infiltrated to the lamina propria, and these were probably reactive lymphocytes. He underwent autologous stem cell transplantation after six cycles of systemic CHOEP chemotherapy. Regular follow-up was done with CT, gastroscopic biopsy and colonofiberscopic biopsy every 3 months. He has been in complete remission for over 300 days after autologous stem cell transplantation.