伴有副肿瘤性白细胞增多的间变大细胞淋巴瘤：5 例临床病理分析。

Anaplastic large cell lymphoma with paraneoplastic leukocytosis: a clinicopathological analysis of five cases.

机构信息

Department of Pathology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.

出版信息

APMIS. 2011 Nov;119(11):794-801. doi: 10.1111/j.1600-0463.2011.02811.x. Epub 2011 Sep 22.

DOI:10.1111/j.1600-0463.2011.02811.x

PMID:21995633

Abstract

Anaplastic large cell lymphoma (ALCL) is a type of T-cell lymphoma with a relatively favorable prognosis. However, a certain group of ALCLs is highly aggressive, featuring paraneoplastic leukocytosis (PL) in clinical presentation. The present study evaluated five cases of ALCL presenting with PL, including four men and one woman, with a median age of 58 years. All cases revealed leukocytosis with a range from 15.3 to 112.9 × 10(3) /μL. Five (100%) and 4 (80%) cases demonstrated immunoreactivity for granulocyte-colony-stimulating factor (G-CSF) and tumor necrosis factor-alpha (TNF-α), respectively. There were significant differences in the expression of G-CSF and TNF-α between ALCL cases with or without PL (p < 0.05 for both). The prognosis of ALCL patients with PL was poor. Four of five patients (80%) died of the disease within a median survival time of 3.5 weeks. The release of G-CSF and TNF-α from lymphoma cells may associate with ALCL presenting with PL, leading to cytokine crisis and even poorer prognosis.

摘要

间变大细胞淋巴瘤（ALCL）是一种 T 细胞淋巴瘤，具有相对较好的预后。然而，某些类型的 ALCL 具有高度侵袭性，在临床表现上伴有副肿瘤性白细胞增多症（PL）。本研究评估了 5 例表现为 PL 的 ALCL 患者，包括 4 名男性和 1 名女性，中位年龄为 58 岁。所有病例均表现为白细胞增多，范围为 15.3 至 112.9×10(3)/μL。5 例（100%）和 4 例（80%）分别对粒细胞集落刺激因子（G-CSF）和肿瘤坏死因子-α（TNF-α）具有免疫反应性。具有或不具有 PL 的 ALCL 病例中 G-CSF 和 TNF-α 的表达存在显著差异（均为 p<0.05）。PL 伴有 ALCL 患者的预后较差。5 例患者中有 4 例（80%）在中位生存时间 3.5 周内死于该疾病。淋巴瘤细胞释放的 G-CSF 和 TNF-α可能与伴有 PL 的 ALCL 相关，导致细胞因子危机，甚至预后更差。