Winkler K, Stührk H, Gaedicke G, Marsmann G, Landbeck G
Klin Padiatr. 1977 Jan;189(8):22-30.
The initial bone marrow smeasrs of 821 children with ALL have been cytochemically analysed. (PAS-, acid phosphatase-(SPH), peroxydase-(POX), alpha-N-esterase-(EST)reaction) with regard to presenting clinical data and course of disease. Granular reactions of PAS and SPH were not meaningful in this respect. The paranuclear SPH-reaction was associated with high incidence of mediastinal mass, high peripheral blas count and male preponderance. All 5 patients with histories of more than 12 weeks duration belongs to the PAS-type of ALL (cloddy reaction). The PAS-type showed a higher incidence of CNS-leukaemia and generally a less favorable prognosis as compared to the UND-type. Thereby a high proportion of PAS positive cells, independently of other risk factors, indicates a better prognosis. The highterto neglected weak EST-reaction (Grad I and II according to Löffler) seems to signalise a clincally distinct subtype of ALL with a younger age distribution maximum as compared to the UND-type, low incidence of risk factors, high rate of remissions lasting more than 2 years but unfavorable endprognosis.
对821例急性淋巴细胞白血病(ALL)患儿的初始骨髓涂片进行了细胞化学分析(采用PAS、酸性磷酸酶(SPH)、过氧化物酶(POX)、α-N-酯酶(EST)反应),并结合临床数据和病程进行分析。PAS和SPH的颗粒反应在这方面无意义。核旁SPH反应与纵隔肿块高发生率、外周血原始细胞计数高及男性优势相关。病程超过12周的所有5例患者均属于ALL的PAS型(块状反应)。与UND型相比,PAS型中枢神经系统白血病发生率较高,总体预后较差。因此,独立于其他危险因素,PAS阳性细胞比例高提示预后较好。此前被忽视的较弱的EST反应(根据Löffler标准为I级和II级)似乎表明ALL存在一种临床上独特的亚型,与UND型相比,其年龄分布高峰更年轻,危险因素发生率低,缓解持续超过2年的比例高,但最终预后不良。