Marbello Laura, Ricci Francesca, Nosari Anna Maria, Turrini Mauro, Nador Guido, Nichelatti Michele, Tedeschi Alessandra, Vismara Eleonora, Morra Enrica
Department of Hematology, Niguarda Ca' Granda Hospital, Milan, Italy.
Leuk Res. 2008 Aug;32(8):1221-7. doi: 10.1016/j.leukres.2008.01.004. Epub 2008 Mar 3.
Hyperleukocytic acute myeloid leukaemia is considered to have a poor prognosis due to high early death rate secondary to leukostasis. Supportive treatments do not seem to have reduced early exitus in this subset of patients. Prognostic impact of hyperleukocytosis on outcome has been the object of few studies. Clinical characteristics and outcome of 45 consecutive adult patients with newly diagnosed acute myeloid leukaemia presenting to our institution with a white cell count (WBC) above 100 x 10(9)L(-1) were reviewed. The outcome of this subset of patients was compared with 200 patients with a leukocyte count lower than 100 x 10(9)L(-1) similarly treated in the same period. Eight hyperleukocytic patients (17%) died of intracranial haemorrhage or pulmonary failure due to leukostasis within the first 7 days of treatment. A significant association was found between complete response (CR) and absence of hyperleukocytosis, but if early deaths were removed from analysis the difference was no longer significant. Hyperleukocytosis also significantly reduces the overall survival (OS) but does not significantly influence the disease-free survival (DFS). We reviewed in literature studies in which the outcome of series of at least 10 patients with hyperleukocytosis were compared with that of patients with a leukocyte count lower than 100 x 10(9)L(-1). Our data were consistent with those of the literature regarding the rate of early mortality and causes of death. In most of the reviewed series hyperleukocytosis does not seem to influence the outcome of patients. Avoiding early death seems to be an important step in this subset of patients. New data about pathophysiology of leukostasis are needed.
高白细胞急性髓系白血病因白细胞淤滞导致的早期死亡率高，被认为预后较差。支持性治疗似乎并未降低该亚组患者的早期死亡率。高白细胞血症对预后的影响很少有研究。我们回顾了45例新诊断的急性髓系白血病成年患者的临床特征和预后，这些患者就诊时白细胞计数（WBC）高于100×10⁹/L⁻¹。将该亚组患者的预后与同期接受类似治疗的200例白细胞计数低于100×10⁹/L⁻¹的患者进行了比较。8例高白细胞患者（17%）在治疗的前7天内因白细胞淤滞死于颅内出血或呼吸衰竭。完全缓解（CR）与无高白细胞血症之间存在显著关联，但如果在分析中排除早期死亡病例，差异不再显著。高白细胞血症也显著降低总生存期（OS），但对无病生存期（DFS）无显著影响。我们回顾了文献中至少10例高白细胞血症患者系列与白细胞计数低于100×10⁹/L⁻¹患者系列预后比较的研究。我们的数据与文献中关于早期死亡率和死亡原因的报道一致。在大多数回顾的系列研究中，高白细胞血症似乎不影响患者的预后。避免早期死亡似乎是该亚组患者的重要一步。需要关于白细胞淤滞病理生理学的新数据。
