Lange Magdalena, Żawrocki Anton, Nedoszytko Bogusław, Wasąg Bartosz, Niedoszytko Marek, Jassem Ewa, Nowicki Roman, Żmijewski Michał A, Biernat Wojciech
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.
Int Arch Allergy Immunol. 2014;165(2):104-10. doi: 10.1159/000368799. Epub 2014 Nov 15.
Neoplastic mast cells involving the bone marrow (BMMCs) of patients with mastocytosis display an aberrant expression of CD25 and/or CD2 antigens. The aim of this study was to determine the frequency of CD2 and CD25 expression on skin mast cells (sMCs) of patients with mastocytosis in the skin at the early stage of the disease. Furthermore, the usefulness of the phenotypic profile of sMCs for the diagnosis of systemic mastocytosis (SM) was evaluated.
The 52 adults included in the study were diagnosed with mastocytosis strictly according to the criteria of the World Health Organization. CD117, CD2 and CD25 antigen expression on sMCs was detected by immunohistochemistry. The presence of the KIT D816V mutation in the BM was analyzed using allele-specific PCR.
The presence of CD2- or CD25-positive sMCs was detected in 57.1% of cutaneous mastocytosis (CM) and 90.3% of SM cases (p = 0.008). In all mastocytosis patients, CD2 expression on sMCs was more frequent than CD25 expression (67.3 and 38.5%, respectively). Moreover, CD2 expression on sMCs was more frequent in SM than in CM cases (p = 0.02). The presence of one of the aberrant sMC antigens was detected in 84.2% of patients with the KIT D816V mutation in the BM. A positive correlation between densities of CD25- and CD117-positive sMCs was found in SM patients (r = 0.46, p = 0.009).
Although sMCs displayed immunoreactivity for one of the neoplastic antigens in the majority of SM patients, the aberrant CD2 and/or CD25 expression on sMCs is not as indicative of SM as the BMMC immunophenotype.
肥大细胞增多症患者骨髓中的肿瘤性肥大细胞(BMMCs)表现出CD25和/或CD2抗原的异常表达。本研究的目的是确定疾病早期皮肤肥大细胞增多症患者皮肤肥大细胞(sMCs)上CD2和CD25表达的频率。此外,评估了sMCs的表型特征对系统性肥大细胞增多症(SM)诊断的有用性。
本研究纳入的52名成年人严格按照世界卫生组织的标准诊断为肥大细胞增多症。通过免疫组织化学检测sMCs上CD117、CD2和CD25抗原的表达。使用等位基因特异性PCR分析BM中KIT D816V突变的存在情况。
在57.1%的皮肤肥大细胞增多症(CM)和90.3%的SM病例中检测到CD2或CD25阳性的sMCs(p = 0.008)。在所有肥大细胞增多症患者中,sMCs上CD2的表达比CD25的表达更常见(分别为67.3%和38.5%)。此外,sMCs上CD2的表达在SM中比在CM病例中更常见(p = 0.02)。在BM中存在KIT D816V突变的患者中,84.2%检测到一种异常的sMC抗原。在SM患者中发现CD25和CD117阳性sMCs的密度之间存在正相关(r = 0.46,p = 0.009)。
虽然在大多数SM患者中sMCs对一种肿瘤抗原表现出免疫反应性,但sMCs上异常的CD2和/或CD25表达并不像BMMC免疫表型那样指示SM。