Jansen J, Hermans J, Remme J, den Ottolander G J, Cardozo P L
Scand J Haematol. 1978 Jul;21(1):60-71.
Hairy-cell leukaemia (leukaemic reticuloendotheliosis) is a well-defined clinical entity. Most of the recent reports are almost entirely concerned with the pathological and functional aspects of the disease. In the present retrospective study the clinical features and laboratory data of 12 patients were analyzed together with a series of 123 adequately clinically documented cases from the literature. The Hb level and the sex of the patient proved to be the only parameters having some prognostic value for the survival time after diagnosis. The effect of splenectomy was assessed in two comparable groups of 24 splenectomized and 51 non-splenectomized patients. The operation seemed to be beneficial, but after 2 years the difference was not significant(.05 less than P less than .10). Analysis of subgroups showed that splenectomy was definitely beneficial in women, in patients with a Hb level over 8.0 g/dl or a platelet level above 50 X 10(9)/1, in patients with leucocytes below 3 X 10(9/1, , and also in patients with hepatomegaly (P less than .05 in all cases). These findings suggest that splenectomy is beneficial in cases where anaemia and thrombocytopenia are not very severe; in severe cytopenia the operation does not increase the life expectancy.
毛细胞白血病(白血病性网状内皮细胞增多症)是一种明确的临床实体。近期的大多数报告几乎完全关注该疾病的病理和功能方面。在本回顾性研究中,分析了12例患者的临床特征和实验室数据,并结合文献中的123例有充分临床记录的病例。患者的血红蛋白水平和性别被证明是对诊断后生存时间具有一定预后价值的唯一参数。在两组可比的患者中评估了脾切除术的效果,一组24例接受脾切除术,另一组51例未接受脾切除术。手术似乎有益,但2年后差异不显著(0.05<P<0.10)。亚组分析表明,脾切除术对女性、血红蛋白水平超过8.0g/dl或血小板水平高于50×10⁹/L的患者、白细胞低于3×10⁹/L的患者以及有肝肿大的患者肯定有益(所有情况下P<0.05)。这些发现表明,在贫血和血小板减少不太严重的情况下,脾切除术是有益的;在严重血细胞减少的情况下,手术不会延长预期寿命。