以全血细胞减少或高白细胞为主要表现的急性白血病的临床分析

Clinical Analysis of Pancytopenic Acute Leukemia or Hyperleukocytic Acute Leukemia

目的 探讨全血细胞减少性急性白血病 (PAL)和高白细胞性急性白血病 (HAL)临床特点。方法 回顾性分析 183例原发急性白血病 (AL)的临床资料。结果 PAL 3 8例 ( 2 0 8% )、HAL 42例 ( 2 3 0 % )。 2 3 0 %的急性髓细胞白血病 (AML) (尤其 5 6 3 %的急性早幼粒细胞白血病 )和 16 9%的急性淋巴细胞白血病 (ALL)表现为PAL ,髓外浸润较轻 ,全血细胞减少明显 ;73 7%的病例可在外周血发现数量不等的白血病细胞 ,早期病死率无明显增加 ,近期和远期疗效与一般类型AL无差别。 2 3 8%的AML(尤其 5 1 2 %急性单核细胞白血病 )和 18 6%的ALL表现为HAL ,肿瘤负荷大 ,髓外浸润重 ,早期病死率高 ,不易获得完全缓解 ,预后恶劣。结论 全血细胞减少或高白细胞是AL患者常见血象表现。了解其特殊血象 ,有利于指导临床诊断、治疗和判断预后。

Objective To explore the clinical characteristics of pancytopenic acute leukemia(PAL) and hyperleukocytic acute leukemia(HAL). Methods 183 patients with primary AL were analyzed retrospectively and divided into three groups according to patients' hemogram. Results Among these patients, 38 cases(20 8%) were PAL(group A), 42 cases(23 0%) were HAL(group B) and 103 cases(56 3%) were generic type(group C). 23 0% cases of acute myeloid leukemia(AML), 56 3% acute promyelocytic leukemia and 16 9% acute lymphoblastic leukemia( ALL) appeared PAL. The manifestations of group A were mainly milder infiltration of extra-bone marrow, more severe cytopenias. The early mortality, short and long-term effectiveness were similar to those of group C. 23 8% AML, 51 2% acute monoblastic leukemia and 18 6% ALL presented HAL. There were higher tumor burden,obviously infiltration of extra-bone marrow, higher early mortality, more refractory treatment, poorer prognosis in group B as compared with group A or C. Conclusions Pancytopenia or hyperleukocytosis were common manifestations in primary AL. It is important to understand these features of patients' hemogram for diagnosis, treatment and prognosis evaluation of leukemia.