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A Case of Congenital T Cell Lymphoblastic Lymphoma

Journal of the Korean Pediatric Society 1994;37(9):1296-1304.
Published online September 15, 1994.
A Case of Congenital T Cell Lymphoblastic Lymphoma
Eun Sun Yoo1, Young Mi Hong1, Kyung Hee Kim1, Hae Soo Gyu2, Eun Chul Chung3
1Department of Pediatrics, Ewha University, College of Medicine, Seoul, Korea
2Department of Anatomical Pathology, Ewha University, College of Medicine, Seoul, Korea
3Department of Radiology, Ewha University, College of Medicine, Seoul, Korea
선천성 T세포형 림프아세포성 림프종 1례
유은선1, 홍영미1, 김경희1, 구혜수2, 정은철3
1이화여자대학교 의과대학 소아과학교실
2이화여자대학교 의과대학 해부병리학교실
3이화여자대학교 의과대학 방사선과학교실
Abstract
T cell lymphoblastic lymphoma is characterized by immature lymphoid cells that are indistinguishable from the lymphoblasts and prolymphocytes of acute lymphoblastic leukemia. Several characteristic clinical features of lymphoblastic lymphoma, with include a high male-to-female ratio, a relatively high incidence in older children and young adults, the frequent presence of mediastinal involvement at the time of diagnosis. Also, this disease is rapidly progressive, and early dissemination to the bone marrow, blood, and central nervous system leads to the evolution of a picture resembling a acute lymphoblastic leukemia. We have experienced a rare case of congeital T-cell lymphoblastic lymphoma in which 1/365 year old female newborn had generalized multiple irregular protruding mass on her body. On bone marrow biops, CSF analysis, ultrasonogram and whole body MRI studies, We found metastasized tumor mass to CNS, orbit, abdominal cavity, bulva, skin, and lower extremities. Histopathologically, specimen from mass on the right thigh showed diffuse infiltration of poorly differentiated and immature lymphoid cells in the skelectal muscle and subcutaneous soft tissue. By immunophenotyping studies using anti T cell and B cell monoclonal antibody, these cells were reactive with UCHL-1, MB2, but unreactive with MB1, L26, Based on these findings, the lesion was diagnosed as lymphoblastic lymphoma of the T-cell type, which occurred congenitally. Most T cell lymphoblastic lymphoma are noted, but congenital case was not reported. So we report it with a brief review of literature.
Key Words: Congenital, T cell lymphoblastic lymphoma


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