Flandrin G, Sigaux F, Castaigne S, Calvo F, Sebahoun G, Clot P
Presse Med. 1984 Dec 22;13(46):2795-9.
Large series of patients with hairy cell leukaemia have only recently been published. The 211 cases reported here provide useful information on the clinical presentation, prognostic factors, evolution and management of this uncommon disease. At presentation, 28% of the patients had no spleen enlargement, and only 20% had severe pancytopenia. However, severe neutropenia (less than 0.5 X 10(9)/l) was present in 32% of the cases. Prognosis was primarily related to the degree of peripheral cytopenia, usually corrected by splenectomy, but it was poor in both non-splenomegalic and splenectomized patients with persistent anaemia and neutropenia, and it is in these patients that other treatments should be tested.
大量毛细胞白血病患者的系列研究直到最近才发表。本文报告的211例病例为这种罕见疾病的临床表现、预后因素、病情发展及治疗提供了有用信息。就诊时,28%的患者无脾肿大,仅有20%的患者有严重全血细胞减少。然而,32%的病例存在严重中性粒细胞减少(低于0.5×10⁹/L)。预后主要与外周血细胞减少程度有关,通常可通过脾切除术纠正,但对于无脾肿大及脾切除术后仍有持续性贫血和中性粒细胞减少的患者,预后较差,正是这些患者应接受其他治疗的检验。