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黑色素瘤黑变病中的三色尿症

Trichochromuria in melanosis of melanoma.

作者信息

Rorsman H, Agrup P, Carlén B, Hansson C, Jonsson N, Rosengren E, Tegner E

出版信息

Acta Derm Venereol. 1986;66(6):468-73.

PMID:2433864
Abstract

Two patients with metastasizing melanoma and diffuse melanosis have previously been reported to excrete large quantities of trichochromes in the urine. The present study describes 2 further melanoma patients with diffuse melanosis and trichochromuria. The hair of one of the patients which had been red in childhood and turned brown in adult age returned to red with the appearance of melanosis. Normal excretion of a methylated melanocytic metabolite, 6-hydroxy-5-methoxyindole-2-carboxylic acid, was observed in this patient, possibly indicating exhaustion of the methylating system. The other patients excreted large quantities of 6-hydroxy-5-methoxyindole-2-carboxylic acid. Both patients showed highly increased excretion of 5-S-cysteinyldopa. Both patients with melanosis exhibited fine electrone-dense granules in lysosomes of dermal histiocytes. The findings support the concept that trichochromes or similar pigments in dermal histiocytes are responsible for diffuse melanosis in melanoma patients.

摘要

此前有报道称,两名患有转移性黑色素瘤和弥漫性黑变病的患者尿液中排出大量三色色素。本研究描述了另外两名患有弥漫性黑变病和三色色素尿症的黑色素瘤患者。其中一名患者的头发在儿童时期是红色的,成年后变成了棕色,随着黑变病的出现又变回了红色。在该患者中观察到甲基化黑素细胞代谢物6-羟基-5-甲氧基吲哚-2-羧酸的正常排泄,这可能表明甲基化系统已耗尽。另一名患者排出大量的6-羟基-5-甲氧基吲哚-2-羧酸。两名患者的5-S-半胱氨酰多巴排泄量均显著增加。两名患有黑变病的患者在真皮组织细胞的溶酶体中均表现出细小的电子致密颗粒。这些发现支持了这样一种观点,即真皮组织细胞中的三色色素或类似色素是黑色素瘤患者弥漫性黑变病的原因。

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