高白细胞性急性白血病 244 例临床分析

244 PATIENTS WITH HYPERLEUKOCYTIC ACUTE LEUKEMIA

为了解高白细胞性急性白血病（ＨＡＬ）的临床规律和预后因素。对２４４例ＨＡＬ和同期非ＨＡＬ进行回顾性对照分析。结果显示：发现ＨＡＬ构成比为８．５％，ＨＡＬ中急性淋巴细胞白血病（ＡＬＬ）较多，在急性髓细胞白血病（ＡＭＬ）中Ｍ５占的比例最多，为４０．５％。ＨＡＬ发病时肝、脾、淋巴结肿大，ＤＩＣ，中枢神经系统白血病（ＣＮＳＬ）较非ＨＡＬ为多。完全缓解（ＣＲ）率为４１．４％，低于非ＨＡＬ的５４．２％，早期病死率是２３．８％。早期死亡的主要原因是颅内出血，占５０％。早期死亡的高危因素有发病时Ｈｂ≤４０ｇ／Ｌ，血小板≤３０×１０９／Ｌ，ＤＩＣ，感染，ＣＮＳＬ。结果显示：ＨＡＬ完全缓解率低，早期死亡率高，预后差，特别是ＨＡＬ－ＡＭＬ的早期死亡率明显高于ＨＡＬ－ＡＬＬ，要重视其早期处理。

To know the clinical characteristics and the prognostic factors of hyperleukocytic acute leukemia, we reviewed 244 patients with acute leukemia associated with hyperleukocytosis. Restrospective analysis and control study were used. Hyperleukocytosis occured in 8.5% of patients with acute leukemia. Hyperleukocytosis in ALL was more common than that in AML. Among AML with hyperleukocytosis, M 5 subtype was the most. Hepatomegaly, splenomegaly, lymphadenopathy, DIC and CNSL were more frequent in hyperleukocytosis group. The complete remission rate was 41.4% for patients with hyperleukocytosis versus 54.2% for patients with non hyperleukocytosis. The early mortality rate was significantly increased in hyperleukocytic patients (23.8%) as compared to the nonhyperleukocytic group (11.1%). Intracranial hemorrhage was the main cause of early death. The high risk factors of early death were: hemoglobin≤40 g/L, blood platelet≤30×10 9/L, DIC, infection, CNSL at presentation. Acute leukemia with hyperleukocytosis has poor prognosis. Especially, acute myeloid leukemia with hyperleukocytosis must be taken seriously because of high early mortality rate.