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具有蕈样肉芽肿表现、对苯妥英超敏反应及淋巴细胞调节异常的假性淋巴瘤

Pseudolymphoma with mycosis fungoides manifestations, hyperresponsiveness to diphenylhydantoin, and lymphocyte disregulation.

作者信息

Rosenthal C J, Noguera C A, Coppola A, Kapelner S N

出版信息

Cancer. 1982 Jun 1;49(11):2305-14. doi: 10.1002/1097-0142(19820601)49:11<2305::aid-cncr2820491118>3.0.co;2-g.

DOI:10.1002/1097-0142(19820601)49:11<2305::aid-cncr2820491118>3.0.co;2-g
PMID:6978761
Abstract

Three patients receiving diphenylhydantoin (DPH) were seen with a reversible process suggesting mycosis fungoides. Clinical and laboratory manifestations included generalized pruritic exfoliative erythroderma, eosinophilia, lymphadenopathy, hepatosplenomegaly, circulating Sézary cells, epidermal Pautrier's microabscesses on skin biopsy, and moderate liver dysfunction. Studies of the distribution and function of the various lymphocyte subpopulations from these patients showed: (1) an increase in the relative and absolute number of T lymphocytes (85--92%); (2) significant stimulation of lymphocyte-blastic transformation by DPH and low response to pokeweed mitogen stimulation; (3) the impaired ability of T gamma lymphocytes to suppress B-cell differentiation and immunoglobulin production. With only one exception, 15 symptom-free patients on DPH showed none of these abnormalities. The clinical manifestations and immunologic abnormalities of patients with this pseudo mycosis fungoides syndrome remitted three to four weeks after DPH administration was discontinued. The proliferation of T lymphocytes and the inhibition of the function of T gamma-suppressor lymphocytes noted in these patients may be significant to the development of other types of pseudolymphoma and to that of true lymphoma.

摘要

三名接受苯妥英(DPH)治疗的患者出现了一个提示蕈样肉芽肿的可逆过程。临床和实验室表现包括全身性瘙痒性剥脱性红皮病、嗜酸性粒细胞增多、淋巴结病、肝脾肿大、循环中的Sezary细胞、皮肤活检显示表皮Pautrier微脓肿以及中度肝功能障碍。对这些患者各种淋巴细胞亚群的分布和功能研究显示:(1)T淋巴细胞的相对和绝对数量增加(85% - 92%);(2)DPH对淋巴细胞母细胞转化有显著刺激作用,对商陆有丝分裂原刺激反应低;(3)Tγ淋巴细胞抑制B细胞分化和免疫球蛋白产生的能力受损。除一例例外,15名服用DPH无症状的患者未出现这些异常。停用DPH三到四周后,这种假性蕈样肉芽肿综合征患者的临床表现和免疫异常消失。这些患者中观察到的T淋巴细胞增殖和Tγ抑制性淋巴细胞功能抑制可能对其他类型假性淋巴瘤和真性淋巴瘤的发生具有重要意义。

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