慢性中性粒细胞白血病：14例罕见骨髓增殖性疾病新病例。
Chronic neutrophilic leukaemia: 14 new cases of an uncommon myeloproliferative disease.
作者信息
Böhm J, Schaefer H E
机构信息
Department of Pathology, University of Freiburg, Medical School, Freiburg, Germany.
出版信息
J Clin Pathol. 2002 Nov;55(11):862-4. doi: 10.1136/jcp.55.11.862.
BACKGROUND
Chronic neutrophilic leukaemia (CNL) is a distinct BCR/ABL negative myeloproliferative disorder of elderly patients characterised by sustained neutrophilia and splenomegaly. The bone marrow shows expansion of neutrophilic granulopoiesis, without excess of myeloblasts. To date, only 129 cases of CNL have been reported in the literature.
AIMS
To report the findings from a large group of 14 new cases of CNL, consisting of eight women and six men (mean age, 64.7 years).
METHODS
A review of the 14 new cases of CNL and the investigation of BCR/ABL translocations in these patients.
RESULTS
Three quarters of the patients died within two years after diagnosis, mostly as a result of severe cerebral haemorrhage. Two younger patients were successfully treated with allogeneic bone marrow transplantation or interferon, which resulted in haematological remission for years.
CONCLUSION
CNL is a rare myeloproliferative disease mostly taking a fatal clinical course, despite the presence of mature neutrophils as leukaemic cells in the blood. Thus, it is important to recognise CNL to develop appropriate therapeutic strategies for affected patients.
背景
慢性中性粒细胞白血病（CNL）是一种独特的、BCR/ABL阴性的骨髓增殖性疾病，多见于老年患者，其特征为持续性中性粒细胞增多和脾肿大。骨髓显示中性粒细胞生成增加，但原始粒细胞无增多。迄今为止，文献中仅报道了129例CNL病例。
目的
报告一组14例新的CNL病例的研究结果，其中包括8名女性和6名男性（平均年龄64.7岁）。
方法
对14例新的CNL病例进行回顾，并对这些患者进行BCR/ABL易位检测。
结果
四分之三的患者在诊断后两年内死亡，主要死于严重脑出血。两名较年轻的患者通过异基因骨髓移植或干扰素成功治疗，实现了多年的血液学缓解。
结论
CNL是一种罕见的骨髓增殖性疾病，尽管血液中的白血病细胞为成熟中性粒细胞，但大多呈致命的临床病程。因此，认识CNL对于为受影响患者制定适当的治疗策略很重要。
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