Isobe Y, Kawamata N, Sato N, Irie S, Kato A, Mori T, Hirano T, Oshimi K
Department of Medicine, Juntendo University School of Medicine.
Rinsho Ketsueki. 1998 Sep;39(9):692-7.
A 72-year-old man was referred to our hospital because of lymphadenopathy, splenomegaly, and leukocytosis. His WBC count was 54,300/microliter, with 89.6% atypical lymphocytes two to three times the diameter of red blood cells, cleaved nuclei, and one or two nucleoli. A lymph node specimen revealed a vaguely nodular pattern, and the diagnosis of mantle cell lymphoma (MCL) was made. The lymphoma cells appeared smaller and more mature than the leukemic cells. The phenotype of the peripheral blood and the lymph node cells was CD5+ CD10- CD19+ CD20+ and the same rearranged JH bands were detected, suggesting that their lymphocytes were of the same origin. In addition, the phenotype of the leukemic cells was CD23+ CD38+ CD43- CD44+ FMC-7+ micro+ chi+. Cytogenetic analysis revealed complex anomalies but not t(11; 14). Cyclin D1 protein was not detected. Because the lymphocyte morphology of the peripheral blood and lymph nodes was discordant, we speculated that variant large cells had proliferated mainly in the peripheral blood. The patient achieved a partial response after 6 courses of CHOP regimen, and was then placed on a COP regimen. He seemed to have MCL, but the following findings were unusual: marked lymphocytosis at initial presentation, discordant morphology, CD5+ CD10- CD23+ CD43- phenotype with neither t(11; 14) or cyclin D1 over-expression.
一名72岁男性因淋巴结病、脾肿大和白细胞增多症被转诊至我院。他的白细胞计数为54300/微升,其中89.6%为非典型淋巴细胞,直径是红细胞的两到三倍,有核裂,并有一到两个核仁。一份淋巴结标本显示出模糊的结节状模式,诊断为套细胞淋巴瘤(MCL)。淋巴瘤细胞看起来比白血病细胞更小且更成熟。外周血和淋巴结细胞的表型为CD5+ CD10- CD19+ CD20+,并且检测到相同的重排JH条带,表明它们的淋巴细胞起源相同。此外,白血病细胞的表型为CD23+ CD38+ CD43- CD44+ FMC-7+ micro+ chi+。细胞遗传学分析显示存在复杂异常,但没有t(11; 14)。未检测到细胞周期蛋白D1蛋白。由于外周血和淋巴结的淋巴细胞形态不一致,我们推测变异的大细胞主要在外周血中增殖。该患者在接受6个疗程的CHOP方案后获得部分缓解,随后接受COP方案治疗。他似乎患有MCL,但以下发现并不常见:初诊时显著淋巴细胞增多、形态不一致、CD5+ CD10- CD23+ CD43-表型,既没有t(11; 14)也没有细胞周期蛋白D1过表达。