Hoyer J D, Ross C W, Li C Y, Witzig T E, Gascoyne R D, Dewald G W, Hanson C A
Department of Laboratory Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Blood. 1995 Aug 1;86(3):1163-9.
We studied 25 T-cell chronic lymphocytic leukemia (T-CLL) cases collected over a 15-year period. Immunophenotypic analysis was performed in each case; 12 cases were evaluated by cytogenetics, and gene rearrangement studies were performed in 14 cases. The median age was 57 years with a male predominance (M:F, 15:10). The median presenting lymphocyte count was 36.3 x 10(9)/L (range, 3.9 to 438 x 10(9)/L). Fourteen patients (56%) had shotty adenopathy and ten (40%) had mild-to-moderate splenomegaly at presentation; four (16%) had erythematous skin lesions. The lymphocytes were predominantly small; some cases had a minor component of medium-sized cells (< 10%). The nuclear: cytoplasmic ratios were uniformly high with round to oval nuclei; however, a wide spectrum of nuclear outlines could be found, ranging from minimally to markedly convoluted. Nucleoli were either absent or small and inconspicuous. These lymphocytes did not have the morphology of prolymphocytes and did not contain cytoplasmic granules. Bone marrow infiltration was generally in an interstitial pattern; the degree of involvement ranged from 15% to 90%. Immunophenotyping showed that the lymphocytes were mature T-cells with a predominant CD4+ immunophenotype. Three cases displayed a CD8+ immunophenotype. The patients were treated with a variety of chemotherapeutic regimens with only a minimal response observed in two of 20 patients. We conclude that T-CLL is an uncommon chronic lymphoproliferative disorder (CLPD) that can be morphologically similar to B-CLL, is distinct from T-prolymphocytic leukemia, and has an aggressive clinical course that is refractory to therapy. It may also be difficult to distinguish T-CLL from other T-CLPD, especially the leukemic phase of peripheral T-cell lymphoma and some cases of Sézary syndrome.
我们研究了在15年期间收集的25例T细胞慢性淋巴细胞白血病（T-CLL）病例。对每例病例进行了免疫表型分析；12例进行了细胞遗传学评估，14例进行了基因重排研究。中位年龄为57岁，男性占优势（男:女，15:10）。初诊时淋巴细胞计数中位数为36.3×10⁹/L（范围为3.9至438×10⁹/L）。14例患者（56%）初诊时有散在淋巴结肿大，10例（40%）有轻至中度脾肿大；4例（16%）有皮肤红斑病变。淋巴细胞主要为小细胞；部分病例有少量中等大小细胞成分（<10%）。核质比均较高，核呈圆形至椭圆形；然而，可发现核轮廓范围广泛，从轻度至明显卷曲。核仁缺如或小且不明显。这些淋巴细胞不具有幼淋巴细胞形态，也不含细胞质颗粒。骨髓浸润一般呈间质型；受累程度为15%至90%。免疫表型分析显示淋巴细胞为成熟T细胞，以CD4⁺免疫表型为主。3例表现为CD8⁺免疫表型。患者接受了多种化疗方案治疗，20例患者中仅2例观察到轻微反应。我们得出结论，T-CLL是一种罕见的慢性淋巴细胞增殖性疾病（CLPD），在形态上可能与B-CLL相似，与T幼淋巴细胞白血病不同，具有侵袭性临床病程且对治疗难治。将T-CLL与其他T-CLPD区分开来也可能困难，尤其是外周T细胞淋巴瘤的白血病期和某些Sezary综合征病例。
