Fisher D E, Jacobson J O, Ault K A, Harris N L
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston 02114.
Cancer. 1989 Nov 1;64(9):1879-87. doi: 10.1002/1097-0142(19891101)64:9<1879::aid-cncr2820640921>3.0.co;2-d.
In patients with diffuse large cell lymphoma (LCL), bone marrow involvement at the time of diagnosis is a poor prognostic sign. Since 1980, the authors have encountered 13 patients LCL who had simultaneous bone marrow involvement by small cleaved cell lymphoma (11 cases) or mixed small and LCL (two cases), a phenomenon known as "discordant" or "divergent" bone marrow histology. The patients ranged in age from 33 to 85 years (median, 61 years) and presented most commonly with Stage III or IV disease, independent of bone marrow involvement. Seventy-seven percent achieved complete remission (CR) with combination chemotherapy; 50% of these eventually relapsed and died of their disease. One patient died of unrelated causes. No recurrences of low-grade lymphoma were observed, as judged either by clinical behavior or rebiopsy. The survival of the patients with discordant bone marrow histology was compared with that of patients with LCL with or without bone marrow involvement by LCL. Of the 11 patients with discordant marrow histology followed for a minimum of 2 years, four (36%) are long-term survivors; this is comparable to the 2-year survival of patients with LCL without bone marrow involvement (45%). In contrast, 89% of patients with bone marrow biopsy specimens positive for LCL died within 18 months from the time of diagnosis (mean survival, 5.7 months). All diffuse LCL tested were of B-lineage. The authors attempted to determine whether the presence of discordant bone marrow histologic types indicated an underlying low-grade B-cell lymphoma in these patients by evaluating the peripheral blood of the long-term survivors for the presence of clonal excess. Of the three surviving evaluable patients tested, one had evidence of clonal excess in the peripheral blood. For patients with LCL who have a simultaneous bone marrow biopsy positive for low-grade lymphoma (discordant marrow histology), survival is no different from that of patients with negative marrows, and markedly better than that for patients with marrows positive for diffuse LCL. The biological significance of discordant bone marrow histology is not clear at this time.
在弥漫性大细胞淋巴瘤（LCL）患者中，诊断时骨髓受累是预后不良的标志。自1980年以来，作者遇到了13例LCL患者，他们同时合并小裂细胞淋巴瘤（11例）或小细胞与LCL混合（2例）累及骨髓，这种现象被称为“不一致”或“不同”的骨髓组织学。患者年龄在33岁至85岁之间（中位年龄61岁），最常见的表现为Ⅲ期或Ⅳ期疾病，与骨髓受累无关。77%的患者通过联合化疗达到完全缓解（CR）；其中50%最终复发并死于疾病。1例患者死于无关原因。根据临床行为或再次活检判断，未观察到低级别淋巴瘤复发。将骨髓组织学不一致的患者生存率与有或无LCL骨髓受累的LCL患者生存率进行了比较。在11例骨髓组织学不一致且至少随访2年的患者中，4例（36%）为长期存活者；这与无骨髓受累的LCL患者2年生存率（45%）相当。相比之下，骨髓活检标本LCL阳性的患者中，89%在诊断后18个月内死亡（平均生存5.7个月）。所有检测的弥漫性LCL均为B细胞系。作者试图通过评估长期存活者外周血中是否存在克隆性异常，来确定骨髓组织学类型不一致是否表明这些患者存在潜在的低级别B细胞淋巴瘤。在检测的3例可评估存活患者中，1例在外周血中有克隆性异常的证据。对于骨髓活检同时显示低级别淋巴瘤阳性（骨髓组织学不一致）的LCL患者，其生存率与骨髓阴性患者无异，且明显优于骨髓弥漫性LCL阳性患者。目前，骨髓组织学不一致的生物学意义尚不清楚。
