BURKITTS-LYMPHOMA DEVELOPING IN A 7-YEAR-OLD BOY WITH HYPER-IGE SYNDROME
BURKITTS-LYMPHOMA DEVELOPING IN A 7-YEAR-OLD BOY WITH HYPER-IGE SYNDROME
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DOI：
10.1016/0091-6749(89)90471-5
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发表时间：
1989-01-01
影响因子：
14.2
通讯作者：
SHEARER, WT
中科院分区：
文献类型：
--
作者：
GORIN, LJ;JEHA, SC;SHEARER, WT
我们报告一例伯基特淋巴瘤发展中的7岁男孩与高IgE综合征。这是高IgE综合征中恶性肿瘤的第三例报告。另外两例为一名18岁男子霍奇金病和一名10岁女孩组织细胞淋巴瘤。患者发生腹膜后伯基特淋巴瘤，可能转移至脑部。他短暂的生命的特点是复发性葡萄球菌皮肤，中耳和肺部感染与极高的血清IgE浓度。还存在与骨质疏松症和病理性骨折相关的骨代谢紊乱以及甲状旁腺素缺乏，这些发现在其他高IgE综合征和其他形式的T细胞免疫缺陷患者中也观察到。在5岁时，在患者中证实了对用源自白喉、破伤风和流感嗜血杆菌B型生物体的抗原和用v φ OX 174噬菌体进行免疫的B细胞应答不足。在这种终末状态下，他的体外淋巴细胞分析显示无反应性。虽然高IgE综合征的确切免疫缺陷尚不清楚，但这些相关恶性肿瘤病例强调了完全正常的免疫系统在预防癌症过早出现中的作用。
We report a case of Burkitt''s lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. This is the third reported case of malignancy in the hyper-IgE syndrome. The other two cases were an 18-year-old man with Hodgkin''s disease and a 10-year-old girl with histiocytic lymphoma. The patient developed retroperitoneal Burkitt''s lymphoma with probable metastasis to the brain. His short life was characterized by recurrent staphylococcal skin, middle ear, and lung infections associated with extremely elevated serum concentrations of IgE. There was also an associated disturbance of bone metabolism with osteoporosis and pathologic fractures and absence of parathormone, findings that have been observed in other patients with hyper-IgE syndrome and other forms of T cell immunodeficiency. At the age of 5 years, inadequate B cell responses to immunization with antigens derived from diphtheria, tetanus, and Haemophilus influenzae type b organisms and with the .vphi.OX174 bacteriophage were demonstrated in the patient. In this terminal state his in vitro lymphocyte analysis demonstrated findings of anergy. Although the precise immunologic defect in hyper-IgE syndrome is unknown, these cases of associated malignancy stress the role that a completely normal immune system plays in preventing the premature appearance of cancer.