Fohlmeister I, Schaefer H E, Mödder B, Hellriegel K P, Fischer R
Blut. 1981 Jun;42(6):367-77. doi: 10.1007/BF00996899.
Seven patients are presented with a chronic lymphoproliferative disorder characterized clinically by splenomegaly, no or discrete lymphnode enlargement, and a varying degree of cytopenia. In blood and bone-marrow smears lymphoid cells of "hairy" appearance are demonstrable which may contain tartrate-resistant acid phosphatase. The finding of a nodular bone-marrow infiltration without fibrosis as well as that of a nodular infiltration of the spleen originating in the white pulp are incompatible with the diagnosis hairy-cell leukemia and place the disease near to chronic lymphocytic leukemia (CLL) or leukemic immunocytoma respectively. A detailed cytologic and cytochemical examination of the infiltrating cells shows deviations from the typical enzymatic pattern of hairy cells and from known enzymatic constellations in CLL and related lymphoproliferative disorders. Thus, we are dealing with an intermediate form, difficult to classify, the separation of which nevertheless seems to be important for therapeutical reasons.
本文报告了7例慢性淋巴细胞增殖性疾病患者,其临床特征为脾肿大,无或仅有散在淋巴结肿大,以及不同程度的血细胞减少。在血液和骨髓涂片上可发现具有“毛发”样外观的淋巴细胞,这些细胞可能含有抗酒石酸酸性磷酸酶。骨髓出现结节状浸润且无纤维化,以及脾脏白髓出现结节状浸润,这些表现均不符合毛细胞白血病的诊断,分别提示该疾病与慢性淋巴细胞白血病(CLL)或白血病性免疫细胞瘤相近。对浸润细胞进行详细的细胞学和细胞化学检查发现,其酶学模式既不同于典型的毛细胞,也不同于CLL及相关淋巴细胞增殖性疾病中已知的酶学组合。因此,我们面对的是一种难以分类的中间形式,不过出于治疗原因,将其区分开来似乎很重要。