Kawano S, Tatsumi E, Yoneda N, Yamaguchi N, Goji J, Ito H, Nagai T, Nishikori M, Okamura A, Koiwai O
Department of Laboratory Medicine, Kobe University School of Medicine, Japan.
Hematol Oncol. 1995 Jan-Feb;13(1):1-11. doi: 10.1002/hon.2900130102.
A 66-year-old male patient was admitted with dyspnea; physical examination revealed petechiae and systemic lymphadenopathy. Laboratory findings showed leukemia. The blasts in the peripheral blood were negative for cytochemical myeloperoxidase, and had condensed nuclear chromatin with a nucleolus. The histological diagnosis of the biopsied neck lymph node was lymphoblastic lymphoma. The leukemia cells expressed CD2, CD6, CD7, CD13low, CD56, beta chain of IL-2 receptorlow (IL-2R beta), and HLA-DR antigens, but not other pan-T (CD5, CD3, CD4, and CD8); pan-B (CD10, CD19, CD20, and CD24); natural killer (NK) (CD16, CD57); or myeloid (CD33) antigens. Electronmicroscopy revealed convoluted nuclei with conspicuous nucleoli and peripherally condensed heterochromatin. Membrane-bound granules containing an electron dense matrix were observed in the cytoplasm, indicating the NK cell nature of the neoplastic cells. While terminal deoxynucleotidyl transferase (TdT) and cytoplasmic CD3 were not detected by immunofluorescence on fixed smears, Northern blot analysis revealed the gene expression of CD3 epsilon, CD3 zeta, and TdT. Gene rearrangement analysis revealed that the beta, gamma, and delta chains of T-cell receptor (TCR) and immunoglobulin heavy chain (IgH) were of germline genotype. While the overall interpretation of the phenotype and genotype was difficult, the derivation of an immature stage of NK lineage was strongly suggested, based predominantly on the electronmicroscopic features. Despite initially successful chemotherapy, the patient died 14 months after initial presentation.
一名66岁男性患者因呼吸困难入院；体格检查发现有瘀点和全身淋巴结病。实验室检查结果显示为白血病。外周血中的原始细胞细胞化学髓过氧化物酶呈阴性，核染色质浓缩，有一个核仁。活检颈部淋巴结的组织学诊断为淋巴母细胞淋巴瘤。白血病细胞表达CD2、CD6、CD7、CD13低表达、CD56、白细胞介素2受体β链（IL-2Rβ）低表达和HLA-DR抗原，但不表达其他全T（CD5、CD3、CD4和CD8）；全B（CD10、CD19、CD20和CD24）；自然杀伤（NK）（CD16、CD57）；或髓系（CD33）抗原。电子显微镜显示核呈卷曲状，有明显的核仁，外周染色质浓缩。在细胞质中观察到含有电子致密基质的膜结合颗粒，表明肿瘤细胞具有NK细胞性质。虽然在固定涂片上通过免疫荧光未检测到末端脱氧核苷酸转移酶（TdT）和细胞质CD3，但Northern印迹分析显示了CD3ε、CD3ζ和TdT的基因表达。基因重排分析显示，T细胞受体（TCR）的β、γ和δ链以及免疫球蛋白重链（IgH）为种系基因型。虽然对表型和基因型的总体解释很困难，但主要基于电子显微镜特征，强烈提示为NK谱系的未成熟阶段。尽管最初化疗成功，但患者在首次就诊后14个月死亡。
