Kaplan J, Cushing B, Chang C H, Poland R, Roscamp J, Perrin E, Bhaya N
Am J Hematol. 1982 May;12(3):247-50. doi: 10.1002/ajh.2830120306.
A family is described in which three of seven siblings developed a T-cell lymphoblastic lymphoma. Tumor cells formed rosettes with sheep erythrocytes, lacked surface Ig, and expressed human T- but not B-lymphocyte antigens. They lacked the enzyme terminal transferase suggesting a lymph node rather than thymic T-cell origin. Autopsy findings supported this conclusion. All three sibs, like their father, had numerous cafe'-au'lait spots indicative of Von Recklinghausen neurofibromatosis. One child had a subcutaneous fibroma, and another had multiple colonic polyps and exostoses characteristic of Gardner syndrome. Both are autosomal dominant conditions known to predispose to malignancies. The genetic factors responsible for these conditions may also predispose to the development of "post-thymic" T-cell lymphoblastic lymphoma.
一个家庭中有7个兄弟姐妹，其中3人患了T细胞淋巴母细胞淋巴瘤。肿瘤细胞与绵羊红细胞形成花环，缺乏表面免疫球蛋白，表达人类T淋巴细胞而非B淋巴细胞抗原。它们缺乏末端转移酶，提示其起源于淋巴结而非胸腺T细胞。尸检结果支持这一结论。所有3个患病的兄弟姐妹，像他们的父亲一样，有许多咖啡牛奶斑，提示冯雷克林霍增氏神经纤维瘤病。一个孩子有皮下纤维瘤，另一个有Gardner综合征特征性的多发结肠息肉和外生骨疣。这两种都是已知易患恶性肿瘤的常染色体显性疾病。导致这些疾病的遗传因素也可能易引发“胸腺后”T细胞淋巴母细胞淋巴瘤的发生。
