Mathé G, Misset J L, Gil-Delgado M, Delgado M, De Vassal F
Recent Results Cancer Res. 1978;65:88-107. doi: 10.1007/978-3-642-81249-1_13.
We have studied 24 cases of secondarily leukemic (stage V) lymphosarcoma (LS), 31 cases of "d'emblée" leukemic LS, and ten cases of lymphoid leukemic neoplasias transitional between "d'emblée" leukemic LS and chronic lymphocytic leukemia (CLL). These cases only concern the common types of the WHO classification of LS, i.e., the prolymphocytic, the lymhoblastic, and the immunoblastic. Some cases have also been classified by cell surface markers. The secondarily leukemic conversion occurred in 40% of the lymphoblastic types, in 14% of the prolymphocytic types, and in 17% of the immunoblastic types. It never occurred at stage I but could occur after any other stage. The mediastinal involvement was observed in three types, but most often in the lymphoblastic type. The prognosis after an acute lymphoid leukemia (ALL) treatment comprising active immunotherapy following chemo(radio)therapy is better for the leukemic prolymphocytic and lymphoblastic LS than for the immunoblastic type. Two patients (one of the lymphoblastic type) are in complete remission after 8 and 5 years, respectively. We have described ten cases of "d'emblée" leukemic LS with either large lymphoid or extra-lymphoid masses, bone marrow leukemic cell involvement, and LS aspects of neoplastic cells. Mediastinal abdominal, or other tumor masses are frequent. The prognosis for "d'emblée" leukemic LS following an ALL treatment is less favorable than ALL prognosis for patients of all ages including children. However, the first remission curve breaks at the 18th month and may form a plateau for about 30% of the patients of all ages. One patient has been in remission for more than 8 years after immunotherapy. We have also described ten cases of lymphoid neoplasia, whose cells cytologically and by the intensity of Ig secretion resemble leukemic prolymphocytic LS cells. However, the disease is more sensitive to CLL treatment than to LS or ALL treatment. Hence, there may be transitional conditions between leukemic LS and CLL. Finally, we have discussed the different possible frontiers between nonleukemic and leukemic LS and proposed two tests to detect the leukemic stage early: the systematic search for LS cells in the peripheral blood after concentration of nucleated cells by centrifugation and for cells carrying immune markers in the isolated mononuclear cell population of peripheral blood and the bone marrow.
我们研究了24例继发性白血病（Ⅴ期）淋巴肉瘤（LS）、31例“初发”白血病性LS以及10例介于“初发”白血病性LS与慢性淋巴细胞白血病（CLL）之间的淋巴样白血病性肿瘤。这些病例仅涉及世界卫生组织LS分类的常见类型，即原淋巴细胞型、淋巴细胞母细胞型和免疫母细胞型。部分病例也通过细胞表面标志物进行了分类。继发性白血病转化在淋巴细胞母细胞型中发生率为40%，在原淋巴细胞型中为14%，在免疫母细胞型中为17%。它从未在Ⅰ期发生，但可在其他任何阶段发生。三种类型均观察到纵隔受累，但最常见于淋巴细胞母细胞型。对于白血病性原淋巴细胞型和淋巴细胞母细胞型LS，在化疗（放疗）后进行主动免疫治疗的急性淋巴细胞白血病（ALL）治疗后的预后优于免疫母细胞型。两名患者（其中一名为淋巴细胞母细胞型）分别在8年和5年后完全缓解。我们描述了10例“初发”白血病性LS，其具有大的淋巴样或淋巴外肿块、骨髓白血病细胞受累以及肿瘤细胞的LS特征。纵隔、腹部或其他肿瘤肿块很常见。“初发”白血病性LS在ALL治疗后的预后不如包括儿童在内的所有年龄段患者的ALL预后。然而，首次缓解曲线在第18个月出现转折，约30%的各年龄段患者可能形成一个平台期。一名患者在免疫治疗后已缓解超过8年。我们还描述了10例淋巴样肿瘤，其细胞在细胞学上以及通过Ig分泌强度类似于白血病性原淋巴细胞型LS细胞。然而，该疾病对CLL治疗比对LS或ALL治疗更敏感。因此，白血病性LS与CLL之间可能存在过渡状态。最后，我们讨论了非白血病性和白血病性LS之间不同的可能界限，并提出了两项早期检测白血病阶段的试验：通过离心浓缩有核细胞后系统检测外周血中的LS细胞，以及检测外周血和骨髓分离的单个核细胞群体中携带免疫标志物的细胞。
