Onishi Yasushi, Matsuno Yoshihiro, Tateishi Ukihide, Maeshima Akiko Miyagi, Kusumoto Masahiko, Terauchi Takashi, Kusumoto Shigeru, Sekiguchi Naohiro, Tanimoto Kazuki, Watanabe Takashi, Kobayashi Yukio, Tobinai Kensei
Hematology Division, National Cancer Center Hospital, Tsukiji, Chou-ku, Tokyo, Japan.
Int J Hematol. 2004 Jul;80(1):43-51. doi: 10.1532/ijh97.04061.
Precursor T-cell lymphoblastic leukemia/lymphoma (T-ALL/LBL) presents a mediastinal mass in one half of cases. Although the immunophenotypic features of T-ALL/LBL have been analyzed in several studies, few studies have been focused on the relationship between the anatomic distribution of lesions and immunophenotypic findings. We analyzed the clinicopathologic findings for 17 patients with T-ALL/LBL diagnosed since 1993 and whose radiologic findings were available. Data on 14 men and 3 women with a median age of 26 years (range, 10-61 years) were analyzed. On the basis of radiologic findings, the cases were divided into thymic type (n = 8) and nonthymic type (n = 9). Patients with the thymic type of T-ALL/LBL had a large mediastinal mass and minimal systemic lymphadenopathy only in the supradiaphragmatic region. Those with the non-thymic type had predominantly systemic lymphadenopathy that included infradiaphragmatic lesions. Expression of CD8 (6/7 versus 0/9) was more frequently found in the thymic type (P < .001), whereas expression of CD56 (0/7 versus 5/9) was more frequent in the nonthymic type (P = .034). In conclusion, T-ALL/LBL was divided into 2 entities, thymic type and nonthymic type, on the basis of radiologic findings and immunophenotypic features. Analysis of the expression of CD8 and CD56 would be useful for biologically classifying T-ALL/LBL into the 2 types. This study was performed in a single institution, was retrospective, and had a limited number of patients; multicenter confirmatory studies are warranted.
前驱T细胞淋巴母细胞白血病/淋巴瘤(T-ALL/LBL)在半数病例中表现为纵隔肿块。尽管已有多项研究分析了T-ALL/LBL的免疫表型特征,但很少有研究关注病变的解剖分布与免疫表型结果之间的关系。我们分析了自1993年以来诊断的17例T-ALL/LBL患者的临床病理结果,且这些患者均有影像学检查结果。分析了14例男性和3例女性的数据,中位年龄为26岁(范围10 - 61岁)。根据影像学检查结果,将病例分为胸腺型(n = 8)和非胸腺型(n = 9)。胸腺型T-ALL/LBL患者有一个大的纵隔肿块,仅在膈上区域有极少的全身淋巴结肿大。非胸腺型患者主要表现为全身淋巴结肿大,包括膈下病变。CD8的表达(6/7对0/9)在胸腺型中更常见(P <.001),而CD56的表达(0/7对5/9)在非胸腺型中更常见(P =.034)。总之,基于影像学检查结果和免疫表型特征,T-ALL/LBL可分为胸腺型和非胸腺型两个实体。分析CD8和CD56的表达有助于将T-ALL/LBL从生物学上分为这两种类型。本研究在单一机构进行,为回顾性研究,且患者数量有限;需要多中心验证性研究。