De Oliveira M S, Jaffe E S, Catovsky D
Department of Haematology and Cytogenetics, Royal Marsden Hospital, London.
J Clin Pathol. 1989 Sep;42(9):962-72. doi: 10.1136/jcp.42.9.962.
Sixteen patients presented with B cell leukaemia (white cell count 26-269 x 10(9)/l) which could not be classified as chronic lymphocytic (CLL), prolymphocytic leukaemia, or follicular lymphoma in leukaemic phase. Eleven patients (10 men, one woman) corresponded histologically to intermediate (INT) or mantle zone lymphoma, and five, with less well defined features, were designated small lymphocytic lymphoma with cleaved cells. The blood films showed a pleomorphic picture with lymphoid cells of predominantly medium size with nuclear irregularities and clefts. The membrane phenotype of the circulating cells showed strong immunoglobulin staining and reactivity with CD5 and FMC7 in all cases tested; CD10 was positive in six out of nine cases. The membrane phenotype of two of the five cases of small lymphocytic lymphoma was close to those of B-CLL and three resembled INT lymphoma. Bone marrow trephine biopsy specimens showed a diffuse pattern of infiltration in INT lymphoma. The median survival of these patients was less than two years, suggesting that a leukaemic presentation is associated with poor prognosis. By combining data from histology, membrane markers, and peripheral blood morphology, the leukaemic phase of typical INT lymphoma can be defined in most cases.
16例患者表现为B细胞白血病（白细胞计数26 - 269×10⁹/L），其无法被归类为慢性淋巴细胞白血病（CLL）、幼淋巴细胞白血病或白血病期滤泡性淋巴瘤。11例患者（10名男性，1名女性）组织学上符合中间型（INT）或套细胞淋巴瘤，5例特征不太明确，被指定为有核裂细胞的小淋巴细胞淋巴瘤。血涂片显示多形性图像，以中等大小的淋巴细胞为主，伴有核不规则和核裂。在所有检测病例中，循环细胞的膜表型显示强免疫球蛋白染色以及与CD5和FMC7反应；9例中有6例CD10呈阳性。5例小淋巴细胞淋巴瘤中有2例的膜表型与B - CLL接近，3例与INT淋巴瘤相似。骨髓活检标本显示INT淋巴瘤呈弥漫性浸润模式。这些患者的中位生存期不到两年，提示白血病表现与预后不良相关。通过结合组织学、膜标志物和外周血形态学数据，大多数情况下可定义典型INT淋巴瘤的白血病期。
