Cuneo A, Balboni M, Piva N, Rigolin G M, Roberti M G, Mejak C, Moretti S, Bigoni R, Balsamo R, Cavazzini P
Institute of Haematology, University of Ferrara, Italy.
Br J Haematol. 1995 Jun;90(2):409-16. doi: 10.1111/j.1365-2141.1995.tb05167.x.
In order to define better the cytological and clinical features of atypical B-cell chronic lymphocytic leukaemia (B-CLL) with t(11:14)(q13;q32), sequential morphologic immunological and cytogenetic studies were performed in seven patients belonging to a series of 72 consecutive cases presenting with a diagnosis of CLL or atypical CLL according to the FAB criteria. Cytologic diagnosis in these seven patients with t(11;14) was typical CLL in two cases presenting with < 10% large lymphocytes (LL) and prolymphocytes (PL) and atypical CLL in five cases in which LL and PL comprised between 10% and 55%. The diagnosis was supported by histologic findings on bone marrow biopsy (five cases) or splenectomy specimens (two cases). A progressive increase of peripheral LL and PL was observed, resulting in a switch of FAB diagnosis over a 6-60-month period from typical CLL into atypical CLL in two cases and from atypical CLL into prolymphocytic leukaemia in five cases. Immunophenotyping showed a mature B-cell phenotype with CD19, CD22, CD24 positivity and CD10 negativity in all patients. A bright-staining pattern for surface immunoglobulins (SIg) was detected in 6/7 cases, CD5 positivity in 6/7 cases, and CD23 positivity in 1/7 cases. The FMC-7 monoclonal antibody was positive in > 40% cells in 5/6 cases. Chromosome changes in addition to t(11;14) were seen in five cases; in two cases unbalanced translocations involving the 3q21 chromosome region, resulting in partial trisomy for the long arm of chromosome 3, were detected early in the course of the disease. Karyotype evolution that was associated with disease progression occurred in 3/6 assessable patients. Comparison of these findings with similar data from 65 B-CLL patients without t(11:14) showed that atypical morphology, switch of FAB diagnosis during the course of the disease, and karyotype evolution were more frequently seen in cases with t(11;14) (5/7 v 15/65 cases, P = 0.015, 7/7 v 7/65 cases, P < 0.0001, and 3/6 v 5/45 assessable cases, P = 0.04, respectively). The frequency of positivity for CD23 and bright SIg staining differed significantly in the two groups. It is concluded that t(11;14) identifies a cytologically atypical subset of B-CLL, characterized by frequent cytologic and cytogenetic evolution and by a distinct immunological profile, sharing some biological features with mantle cell lymphoma.
为了更准确地界定伴有t(11;14)(q13;q32)的非典型B细胞慢性淋巴细胞白血病（B-CLL）的细胞学和临床特征，我们对72例连续病例中符合FAB标准诊断为CLL或非典型CLL的7例患者进行了序贯形态学、免疫学和细胞遗传学研究。这7例伴有t(11;14)的患者中，2例小淋巴细胞（LL）和原淋巴细胞（PL）比例<10%，细胞学诊断为典型CLL；5例LL和PL比例在10%至55%之间，诊断为非典型CLL。骨髓活检（5例）或脾切除标本（2例）的组织学检查结果支持上述诊断。观察到外周血LL和PL逐渐增加，导致6至60个月内，2例患者的FAB诊断从典型CLL转变为非典型CLL，5例患者从非典型CLL转变为原淋巴细胞白血病。免疫表型分析显示，所有患者均为成熟B细胞表型，CD19、CD22、CD24阳性，CD10阴性。7例中有6例检测到表面免疫球蛋白（SIg）呈明亮染色模式，7例中有6例CD5阳性，7例中有1例CD23阳性。6例中有5例FMC-7单克隆抗体在>40%的细胞中呈阳性。除t(11;14)外，5例还发现了染色体改变；2例在疾病早期检测到涉及3q21染色体区域的不平衡易位，导致3号染色体长臂部分三体。6例可评估患者中有3例出现与疾病进展相关的核型演变。将这些结果与65例不伴有t(11;14)的B-CLL患者的类似数据进行比较，结果显示，非典型形态、疾病过程中FAB诊断的转变以及核型演变在伴有t(11;14)的病例中更为常见（分别为5/7例对15/65例，P = 0.015；7/7例对7/65例，P < 0.0001；3/6例对5/45例可评估病例，P = 0.04）。两组中CD23阳性率和明亮SIg染色频率差异显著。结论是，t(11;14)可识别出B-CLL的一个细胞学非典型亚组，其特征为频繁的细胞学和细胞遗传学演变以及独特的免疫表型，与套细胞淋巴瘤具有一些共同的生物学特征。
