Hill W, Kettner G, Burkhardt R
Klin Wochenschr. 1987 Jul 15;65(14):691-8. doi: 10.1007/BF01875508.
During 1966 and 1986 202 patients with hairy cell leukemia in the bone marrow biopsy were observed. All hairy cells were categorized according to their nuclear morphology into ovoid subtype, convoluted subtype and indented subtype. The overall survival of 113 non-splenectomised patients was 11 months. However, the median survival time of each subtype differed considerably with 55 months for the ovoid subtype, 8 months for the convoluted and 6 months for the indented type. The prognosis of splenectomy patients were significantly better at a p-value of less than 0.0001 than the survival of the non-splenectomised patients. The median survival time was 59 months. Also in this group of patients the prognosis was different for the ovoid, convoluted and indented subtype. The median survival time of the ovoid subtype is not yet reached, despite a observation time of more than 180 months. In contrast, the survival time for the convoluted and indented subtypes was 26 months. These data elucidate that at least part of the heterogeneity in the clinical course of this disease can be explained by the morphologically distinct subtypes and that splenectomy prolongs profoundly but to a different degree the survival of patients with all three histological subtypes. The response rate to r.-IFN alpha-2b is higher in patients with the ovoid than with the convoluted and indented subtypes.
在1966年至1986年期间,对202例经骨髓活检确诊为毛细胞白血病的患者进行了观察。所有毛细胞均根据其核形态分为卵圆形亚型、卷曲亚型和凹陷亚型。113例未行脾切除术患者的总生存期为11个月。然而,各亚型的中位生存期差异显著,卵圆形亚型为55个月,卷曲亚型为8个月,凹陷亚型为6个月。脾切除术患者的预后明显好于未行脾切除术的患者,p值小于0.0001。中位生存期为59个月。在这组患者中,卵圆形、卷曲和凹陷亚型的预后也有所不同。尽管观察时间超过180个月,但卵圆形亚型的中位生存期仍未达到。相比之下,卷曲亚型和凹陷亚型的生存期为26个月。这些数据表明,该疾病临床病程中的至少部分异质性可由形态学上不同的亚型来解释,并且脾切除术可显著延长所有三种组织学亚型患者的生存期,但延长程度不同。卵圆形亚型患者对重组干扰素α-2b的反应率高于卷曲亚型和凹陷亚型患者。