Hepatosplenic αβ T-cell lyrnphomas: A report of 14 cases and comparison with hepatosplenic γδ T-cell lymphomas

Hepatosplenic αβ T-cell lyrnphomas: A report of 14 cases and comparison with hepatosplenic γδ T-cell lymphomas
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肝脾αβT细胞淋巴瘤14例报告并与肝脾γδT细胞淋巴瘤比较

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发表时间:
2001
期刊:
影响因子:
--
通讯作者:
J. Cousar
J. Cousar
中科院分区:
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文献类型:
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作者:
W. Macon;N. Levy;Paul J. Kurtin;K. E. Salhany;M. Elkhalifa;T. T. Casey;F. Craig;C. Vnencak;M. Gulley;Jonathan P. Park;J. Cousar

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肝脾γ - t细胞淋巴瘤是一种独特的实体,其特点是发生在年轻成年男性,伴有肝脾肿大,b症状,外周血细胞减少,无淋巴结病变;淋巴瘤浸润脾红髓、肝窦、骨髓窦;t细胞受体(TCR) γ - δ链和细胞毒性t细胞表型等臂染色体7问;还有一个积极的临床过程。相比之下,本研究描述了14例表达TCR α -链的肝脾t细胞淋巴瘤的临床病理特征。他们发生在11名女性和3名男性中位年龄为36岁。临床表现与先前描述的肝脾γ - t细胞淋巴瘤相似,除了女性优势和年龄分布(5例13岁以下患者和5例50岁以上患者)。疾病主要分布在脾红髓和肝窦,尽管4例肝脏浸润主要在门静脉周围。8例患者骨髓受累,通常发生在间质和/或鼻窦内。5例患者淋巴结受累,但仅有2例可见淋巴结病变。10例为中等大小的肿瘤细胞,核圆/卵圆形,染色质微分散,核仁不明显,细胞质少至中等。4个淋巴瘤主要包括大细胞,细胞核不规则,染色质分散,核仁可见,细胞质中等到丰富。14例淋巴瘤的肿瘤细胞均为细胞毒性α - t细胞;13例共表达自然杀伤细胞相关抗原,显示t细胞克隆性。3例淋巴瘤与eb病毒有关。4例中有2例具有同工染色体7q。11名患者死亡,8名患者在诊断后一年内死亡,2名患者在联合化疗后保持完全缓解。这些数据表明肝脾t细胞淋巴瘤包括α - β亚型。这一组,连同先前认识到的伽马δ组,应该被认为是同一疾病实体的表型异质性亚型。
Hepatosplenic gammadelta T-cell lymphoma is a distinct entity, characterized by occurrence in young adult males with hepatosplenomegaly, B-symptoms, peripheral blood cytopenias, and no lymphadenopathy; lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses; T-cell receptor (TCR) gammadelta chains and a cytotoxic T-cell phenotype; isochromosome 7q; and an aggressive clinical course. In comparison, this study describes the clinicopathologic features of 14 hepatosplenic T-cell lymphomas expressing TCR alphabeta chains. They occurred in 11 women and 3 men with a median age of 36 years. Clinical presentation was similar to that described previously for hepatosplenic gammadelta T-cell lymphomas, except for the female preponderance and age distribution (5 patients younger than 13 years of age and 5 patients older than 50 years of age). Disease distribution was primarily in the splenic red pulp and hepatic sinusoids, although liver infiltrates were largely periportal in four cases. Bone marrow involvement, observed in eight patients, was usually interstitial and/or within the sinuses. Lymph nodes were involved in five patients, although lymphadenopathy was demonstrable in only two. Ten cases were composed of intermediate-size tumor cells with round/oval nuclei, slightly dispersed chromatin, inconspicuous nucleoli, and scant to moderate amounts of cytoplasm. Four lymphomas contained primarily large cells with irregular nuclei, dispersed chromatin, discernible nucleoli, and moderate to abundant cytoplasm. Tumor cells in all 14 lymphomas were cytotoxic alphabeta T-cells; 13 co-expressed natural killer cell-associated antigens and showed T-cell clonality. Three lymphomas were associated with Epstein-Barr virus. Two of four cases had an isochromosome 7q. Eleven patients are dead, eight within a year of diagnosis, and two patients have maintained complete remissions after combination chemotherapy. These data show that hepatosplenic T-cell lymphomas include an alphabeta-subtype. This group, along with the previously recognized gammadelta group, should be recognized as phenotypically heterogeneous subtypes of the same disease entity.
DOI: 10.1182/blood.v82.1.1.bloodjournal8211
发表时间: 1993-07
期刊: Blood
影响因子: 20.3
作者:
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通讯作者: T. Loughran
人类 T γ δ 细胞的组织定位和 CD8 辅助分子表达。
DOI: --
发表时间: 1989
期刊: Journal of immunology (Baltimore, Md. : 1950)
影响因子: --
作者:
Bucy,RP;Chen,CL;Cooper,MD
通讯作者: Cooper,MD
γ/δ T 细胞移植后淋巴增殖性疾病主要发生在脾脏。
DOI: 10.1093/ajcp/102.3.310
发表时间: 1994
影响因子: 3.5
作者:
Ross,CW;Schnitzer,B;Sheldon,S;Braun,DK;Hanson,CA
通讯作者: Hanson,CA
自然杀伤样 T 细胞淋巴瘤:T 大颗粒淋巴细胞的侵袭性淋巴瘤。
DOI: --
发表时间: 1996
期刊: Blood
影响因子: 20.3
作者:
Macon,WR;Williams,ME;Greer,JP;Hammer,RD;Glick,AD;Collins,RD;Cousar,JB
通讯作者: Cousar,JB