Hepatosplenic T-cell lymphoma of αβ lineage in a 16-year-old boy presenting with hemolytic anemia and thrombocytopenia
Hepatosplenic T-cell lymphoma of αβ lineage in a 16-year-old boy presenting with hemolytic anemia and thrombocytopenia
复制标题
一名 16 岁男孩出现溶血性贫血和血小板减少症的 αβ 系肝脾 T 细胞淋巴瘤
DOI：
10.1097/00000478-200003000-00016
复制
发表时间：
2000
期刊：
影响因子：
--
通讯作者：
R. Coupland
中科院分区：
文献类型：
--
作者：
R. Lai;L. Larratt;W. Etches;S. Mortimer;L. Jewell;L. Dabbagh;R. Coupland
作者报告一例罕见的外周T细胞淋巴瘤，发生于一名16岁男孩，最初表现为黄疸、脾肿大、贫血和血小板减少。一个淋巴瘤被发现随后在脾脏，这是广泛的浸润在红髓的中型，blastic-appearing淋巴瘤细胞。这些细胞的免疫学特征显示CD 3、CD 5、CD 45 RO、CD 56和T细胞胞内抗原（TIA）阳性，而CD 2、CD 3、CD 4、CD 8、CD 57、CD 34和末端脱氧核苷酸转移酶（TdT）阴性。传统的细胞遗传学研究揭示了等染色体7 q的存在。随访时，该患者病情迅速恶化，有肝脏和骨髓受累的证据。虽然这种疾病的总体临床和病理特征是肝脾γ δ T细胞淋巴瘤的特征，但这种肿瘤的T细胞受体显示出α而不是γ δ谱系的免疫表型。使用Southern印迹技术，作者证明了T细胞受体β链的单克隆基因重排。因此，他们证实了肝脾α T细胞淋巴瘤的存在。鉴于其与肝脾γ δ T细胞淋巴瘤的总体相似性，这种不寻常的实体可能代表了同一疾病的轻微生物学变异。
The authors report an unusual case of peripheral T-cell lymphoma in a 16-year-old boy who presented initially with jaundice, splenomegaly, anemia, and thrombocytopenia. A lymphoma was found subsequently in the spleen, which was infiltrated extensively in the red pulp by medium-sized, blastic-appearing lymphoma cells. Immunologic characterization of these cells revealed positivity for CD3, CD5, CD45RO, CD56, and T-cell intracellular antigen (TIA), and negativity for CD2, CD3, CD4, CD8, CD57, CD34, and terminal deoxynucleotidyl transferase (TdT). Conventional cytogenetic studies revealed the presence of isochromosome 7q. On follow up, this patient deteriorated rapidly, with evidence of liver and bone marrow involvement. Although the overall clinical and pathologic features of this disease were characteristic of hepatosplenic gammadelta T-cell lymphoma, the T-cell receptor of this tumor showed an immunophenotype of alphabeta not gammadelta lineage. Using the Southern blot technique, the authors demonstrated monoclonal gene rearrangement of the T-cell receptor beta-chain. Thus, they confirmed the existence of hepatosplenic alphabeta T-cell lymphoma. In view of its overall similarity to hepatosplenic gammadelta T-cell lymphoma, this unusual entity probably represents a slight biologic variation of the same disease.
影响因子：
20.3
作者：
T. Loughran
通讯作者：
T. Loughran
影响因子：
20.3
作者：
A. Scott;David;Head;K. Kopecky;Frederick;Appelbaum;K. Theil;Michael;Grever;I. Chen;Michael H. Whittaker;B. Griffith;J. Licht;S. Waxman;M. Whalen;A. Bankhurst;L. Richter;T. Grogan;Cheryl L. Willrnan
通讯作者：
A. Scott;David;Head;K. Kopecky;Frederick;Appelbaum;K. Theil;Michael;Grever;I. Chen;Michael H. Whittaker;B. Griffith;J. Licht;S. Waxman;M. Whalen;A. Bankhurst;L. Richter;T. Grogan;Cheryl L. Willrnan
CD3、CD56 大颗粒淋巴细胞白血病的侵袭性变体。
影响因子：
20.3
作者：
Gentile,TC;Uner,AH;Hutchison,RE;Wright,J;Ben-Ezra,J;Russell,EC;LoughranJr,TP
通讯作者：
LoughranJr,TP