[T细胞幼淋巴细胞白血病的皮肤定位]

[Cutaneous localization of T-cell prolymphocytic leukemia].

作者信息

Thomas A, Dompmartin A, Troussard X, Moreau A, Manard C, Leroy D

机构信息

Service de Dermatologie, CHU, Caen.

出版信息

Ann Dermatol Venereol. 1995;122(8):526-9.

PMID:8572493

Abstract

INTRODUCTION

T cell prolymphocytic leukemia (T PLL) is a rare variant of mature lympho-proliferative disorder. The main physical sign is a gross splenic enlargement contrasting with no enlargement of lymph nodes. Skin involvement is found in 30 p. 100 cases. Twenty-one cases of cutaneous lesions of PLL have been reported, mainly with T PLL, only 2 cases of B PLL: Clinical lesions are polymorphous; histology shows a dermal prolymphocytic infiltrate. The main cytogenetic abnormalities are: translocation (14; 14) (q11; q32), inversion of chromosome 14 (q11; q32), isochromosome 8q.

CASE REPORT

We report a case of an 87-year-old patient presenting a T cell prolymphocytic leukemia CD4+ with specific papular lesions of the back. Electron microscopy showed typical prolymphocytes and cytogenetic studies showed a tendency to polyploidy, with the lost of chromosome 14, translocation 8-22 and inversion of chromosome 13. After 12 months of treatment with a combination of chloraminophen and prednisone the patient was in partial remission and the cutaneous lesions disappeared.

DISCUSSION

This case is rare and the patient has an unusual long survival (mean survival is 7 months). Contrary to the other hematologic disorders, cutaneous involvement does not change the prognosis of T PLL:

摘要

引言

T 细胞原淋巴细胞白血病（T PLL）是成熟淋巴细胞增殖性疾病的一种罕见变体。主要体征是脾脏明显肿大，而淋巴结无肿大。100 例中有 30 例出现皮肤受累。已报道 21 例 PLL 皮肤病变，主要为 T PLL，仅 2 例为 B PLL：临床病变多样；组织学显示真皮有原淋巴细胞浸润。主要的细胞遗传学异常包括：易位（14；14）（q11；q32）、14 号染色体倒位（q11；q32）、8q 等臂染色体。

病例报告

我们报告一例 87 岁患者，患有 CD4+T 细胞原淋巴细胞白血病，背部有特异性丘疹病变。电子显微镜显示典型的原淋巴细胞，细胞遗传学研究显示有倾向于多倍体的情况，伴有 14 号染色体缺失、8 - 22 易位和 13 号染色体倒位。在用氯胺苯砜和泼尼松联合治疗 12 个月后，患者部分缓解，皮肤病变消失。