Quintanilla-Martinez L, Zukerberg L R, Harris N L
Department of Pathology, Massachusetts General Hospital, Boston 02114.
Am J Surg Pathol. 1992 Nov;16(11):1075-84. doi: 10.1097/00000478-199211000-00006.
The clinical, histologic, and immunohistologic features of three cases of pre-T-cell (CD7+/CD2-) lymphoblastic lymphoma in adults are reported. The patients were adults over age 50 years who had a relatively indolent nodal disease, partial involvement of lymph nodes, and primitive immunophenotype. The phenotype of the three cases was TdT+, HLA-DR+, CD34+, CD71+, CD38+, and CD7+, most resembling the normal prothymocyte, and in contrast to normal thymocytes, which generally coexpress CD1+, CD4+, and CD8+. The prethymic T-cell character was further supported by germline T-cell receptor beta and gamma chain genes. In contrast to most reported cases with this early immunophenotype, these patients had nodal disease but not peripheral blood involvement. Two of the three cases were associated with Langerhans' cell histiocytosis (histiocytosis X), a previously unreported association. Because of the Langerhans' cell histiocytosis and the relatively indolent clinical presentation, the differential diagnosis in all cases included both a benign process and a lower-grade lymphoma. Recognition of this unusual form of adult lymphoblastic lymphoma is essential for correct diagnosis and treatment.
报告了3例成人前T细胞（CD7+/CD2-）淋巴母细胞淋巴瘤的临床、组织学和免疫组织学特征。患者均为50岁以上成人，患有相对惰性的淋巴结疾病，淋巴结部分受累，且具有原始免疫表型。3例病例的表型为TdT+、HLA-DR+、CD34+、CD71+、CD38+和CD7+，最类似于正常前胸腺细胞，与通常共表达CD1+、CD4+和CD8+的正常胸腺细胞相反。种系T细胞受体β和γ链基因进一步支持了前胸腺T细胞特征。与大多数报道的具有这种早期免疫表型的病例不同，这些患者有淋巴结疾病，但无外周血受累。3例病例中有2例与朗格汉斯细胞组织细胞增多症（组织细胞增多症X）相关，这是一种以前未报道过的关联。由于存在朗格汉斯细胞组织细胞增多症以及相对惰性的临床表现，所有病例的鉴别诊断均包括良性过程和低级别淋巴瘤。认识到这种不寻常形式的成人淋巴母细胞淋巴瘤对于正确诊断和治疗至关重要。
