Osmanov D Sh, Kruglova G V, Frenkel' M A, Tupitsin N N, Probatova N A, Kondrat'eva T T, Sholokhova E N, Finogenova I A, Dvoretskiĭ V V
Ter Arkh. 1996;68(7):42-8.
The paper presents clinical, hematological, morphological and immunological characteristics of B-cell lymphosarcoma with prolymphocytic-lymphocytic type of leukemization in 50 adult patients (9 females and 41 males aged 29-86 years). In B-cell immunological subvariant of prolymphocytic-lymphocytic leukemization changes in the primary tumor always corresponded to prolymphocytic variant of lymphosarcoma. This distinguishes B-cell lymphosarcomas from previously described T-cellular ones in which the type of eventual leukemic changes did not always correspond to the kind of initial tumor. The presence or absence of prolymphocytes with split nuclei in bone marrow puncture samples was neither of clinical nor of prognostic significance. In leukemization of B-cell prolymphocytic lymphosarcoma from the cells with split nuclei or cells with different configuration of the nuclei, immunological phenotype typical for B-cell chronic lymphoid leukemia did not occur. In prolymphocytic lymphosarcoma from cells with round nuclei one-third of patients had immunological phenotype more typical for B-cell chronic lymphoid leukemia. However, among them were patients with aggressive course with predominant extranodal location of tumor and prolymphocytic type of leukemization. Tumor nodes in B-cell prolymphocytic lymphosarcomas, irrespective of leukemization morphological variant, proved rather resistant to therapy. A complete clinicohematological remission according to the international criteria occurred in 2 of 50 patients, only.
本文介绍了50例成年患者(9例女性,41例男性,年龄29 - 86岁)具有原淋巴细胞 - 淋巴细胞白血病化类型的B细胞淋巴瘤的临床、血液学、形态学和免疫学特征。在原淋巴细胞 - 淋巴细胞白血病化的B细胞免疫亚变体中,原发性肿瘤的变化始终与淋巴瘤的原淋巴细胞变体相对应。这将B细胞淋巴瘤与先前描述的T细胞淋巴瘤区分开来,在T细胞淋巴瘤中,最终白血病变化的类型并不总是与初始肿瘤的类型相对应。骨髓穿刺样本中有无核分裂的原淋巴细胞既无临床意义也无预后意义。在具有核分裂的细胞或核形态不同的细胞导致的B细胞原淋巴细胞淋巴瘤白血病化过程中,未出现B细胞慢性淋巴细胞白血病典型的免疫表型。在具有圆形核的细胞导致的原淋巴细胞淋巴瘤中,三分之一的患者具有更典型的B细胞慢性淋巴细胞白血病免疫表型。然而,其中包括病程侵袭、肿瘤主要位于结外且为原淋巴细胞白血病化类型的患者。B细胞原淋巴细胞淋巴瘤的肿瘤结节,无论白血病化形态变体如何,对治疗都相当耐药。按照国际标准,50例患者中仅2例实现了完全临床血液学缓解。