Kersey J H, Gajl-Peczalska K J, Coccia P F, Nesbit M E
Am J Pathol. 1978 Feb;90(2):487-95.
One hundred consecutive newly diagnosed cases of leukemia and lymphoma in children from 0 to 16 years of age presenting at the University of Minnesota from 1973 to 1977 were studied. Clinical features were correlated with phenotypic features of blast cells, including surface markers and cytomorphology. Four groups with distinct clinical and pathologic features emerged from the study: a) The acute leukemias of the "null" or "undifferentiated" group were those in which the malignant cells carried distinctive null leukemia surface antigen and lacked features of either T cells (E-rosette positivity) or B cells (surface immunoglobulin positivity). These cases occurred most frequently in the series (56% of total cases), peaked in incidence at 6 years, were associated with extensive bone marrow involvement, lacked distinguishing cytomorphologic features, and had the best response to therapy of all groups. b) The acute myelogenous leukemias, including those with myeloid, monocytoid, or erythroid features or a combination of the above, had extensive bone marrow involvement and the characteristic morphology. This group was seen with intermediate frequency and showed an intermediate response to therapy. c) Leukemia-lymphomas of the T-cell group were frequently associated with mediastinal masses and other masses, a cytomorphology which was different from the B-cell group but similar to the null group, and high white cell counts. These cases occurred with intermediate frequency (14%) and had a worse prognosis than the null group. d) Leukemia-lymphomas of the B-cell group had monoclonal surface immunoglobulin with mu-heavy and either kappa or lambda light chain. These patients were least frequent in the series, frequently presented with abdominal masses, and had a characteristic Burkitt cell morphology. Prognosis was the worst of all patients in our series. These data suggest that the major phenotypic groups of childhood leukemia and lymphoma have differing prognoses and should receive differing forms of therapy. Clinical and pathologic features of each group are sufficiently distinctive to suggest that they may have different causes.
对1973年至1977年在明尼苏达大学就诊的100例0至16岁儿童新诊断的白血病和淋巴瘤病例进行了研究。临床特征与原始细胞的表型特征相关，包括表面标志物和细胞形态学。该研究出现了四组具有不同临床和病理特征的病例：a）“无”或“未分化”组的急性白血病，其恶性细胞携带独特的无白血病表面抗原，且缺乏T细胞（E玫瑰花结阳性）或B细胞（表面免疫球蛋白阳性）的特征。这些病例在该系列中最为常见（占总病例的56%），发病率在6岁时达到峰值，与广泛的骨髓受累相关，缺乏明显的细胞形态学特征，并且在所有组中对治疗的反应最佳。b）急性髓细胞白血病，包括具有髓样、单核细胞样或红系特征或上述特征组合的病例，有广泛的骨髓受累和特征性形态。该组出现频率中等，对治疗的反应也中等。c）T细胞组的白血病 - 淋巴瘤常与纵隔肿块和其他肿块相关，其细胞形态学与B细胞组不同但与无组相似，且白细胞计数高。这些病例出现频率中等（14%），预后比无组差。d）B细胞组的白血病 - 淋巴瘤具有带有μ重链以及κ或λ轻链的单克隆表面免疫球蛋白。这些患者在该系列中最少见，常表现为腹部肿块，并且具有特征性的伯基特细胞形态。预后是我们系列中所有患者中最差的。这些数据表明，儿童白血病和淋巴瘤的主要表型组预后不同，应接受不同形式的治疗。每组的临床和病理特征足够独特，表明它们可能有不同的病因。
