Abstract
Three siblings developed a similar type of lymphoblastic lymphoma. Like their father, they all had multiple cafe au lait spots, and one was found at autopsy to have intestinal polyps typical of Gardner's syndrome, a condition with a known dominant inheritance of malignancies. HL-A type in 2/2 affected children tested was 9, 17/W28, W5. All three children presented with cervical and/or mediastinal lymphomas and died 1-8 months after diagnosis of central nervous system metastasis.
Tumor cells from 2/2 tested were T lymphoblasts. They formed rosettes with sheep erythrocytes, lacked surface Ig and reacted with heterologous anti-T but not anti-B cell serum. In the one child so tested, lymphoblasts lacked the enzyme terminal transferase indicating a lymph node rather than thymic T cell origin. Moreover, unlike children with thymic lymphoblastic lymphoma who usually progress to or have at diagnosis an associated T cell leukemia, none of these three children developed bone marrow involvement. We conclude that these children had a tumor which appears to be a post-thymic T cell lymphoma, and that a genetic predisposition for this tumor occurs in Gardner's syndrome.
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Cushing, B., Hochang, C., Roskamp, J. et al. 593 POST-THYMIC T CELL LYMPHOMA: OCCURRENCE IN THREE SIBLINGS AND ASSOCIATION WITH GARDNER'S SYNDROME. Pediatr Res 12 (Suppl 4), 462 (1978). https://doi.org/10.1203/00006450-197804001-00598
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DOI: https://doi.org/10.1203/00006450-197804001-00598