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[成人中枢神经系统白血病]

[Neuroleukemia in adults].

作者信息

Beslać-Bumbasirević Lj, Bosković D, Tomin D, Colović M, Kovacević M, Bumbasirević V

机构信息

Department of Neurology, Clinical Centre of Serbia, Belgrade.

出版信息

Srp Arh Celok Lek. 1996 Mar-Apr;124(3-4):82-6.

PMID:9102825
Abstract

Involvement of CNS with leukemic cells is well recognized complication of acute lymphatic leukemia (ALL) in childhood, but with recent improvements in systemic treatment and longer survival the incidence of this complication has increased in adults. Neurological symptomatology in patients with CNS leukemia is due to meningeal infiltration, but sometimes also to diffuse and nodular cerebral infiltration. Between January 1991 and December 1994, 36 patients suffering of acute leukemia, 28 with ALL, and 8 with acute myeloid leukemia (AML) were demonstrated to have neuroleukemia by the following criteria: (1) the presence at lumbar puncture (LP) pleocytosis and blast cells on CSF sediment (without positive bacteriologic and fungal cultures), and (2) the presence of neurological symptoms and signs. All 36 patients had 46 episodes of CNS involvement. All patients had neurological examinations during every episode, and according to the neurological abnormalities were classified into four categories. LP was performed in all, and CSF sediments obtained by sedimentation in Sayk's chambers, were routinely stained by MGG and cytochemical stains to detection of leukemic cells (Fig. 1). EEG was done during 21 episodes, CT scan during 15. We divided patients into four groups according to the most prominent neurological symptoms and signs. First was the group included 23 episodes (50%), (18 ALL, 5 AML), where symptoms and signs of meningeal irritation predominated, mimicking the clinical picture of meningitis. This meningeal syndrome can sometimes produce differential diagnostic problems with CNS infections, when CSF examination is of primary importance. Second was the group of 9 patients (6 ALL and 3 AML) with 10 episodes (21.74%) where cranial nerve symptoms and signs-predominated, or were exclusively present. Most frequently affected were bulbomotors, facials and opticus. Third group consisted of 8 ALL patients (8 episodes, 17.39%) with dominant spinal root symptomatology, caused by pathological infiltration of either spinal roots or meninges surrounding them. This group includes also one patient with mononeuritis multiplex and the other with painful polyneuropathy. All patients in this group had pain on straight leg raising, but we stress here that all patients from other groups had positive Lazarevitsh's sign, too. So, it can be a good differential diagnostic parameter for distinguishing toxic medicamentous polyneuropathy from leukemic poliradiculoneuropathy. Fourth group included 5 patients (5 episodes, 10.87%). 4 ALL and 1 AML, where cerebral symptoms, such as seizures, hemiparesis and psychoorganic syndromes were prominent. CSF was obtained during all episodes by lumbar puncture. The protein concentration ranged from 21-3180 mg/dl, and was above normal (45 mg/dl) during 28 episodes. Mild hypoglycoracchia was present during 16 episodes. Cell count ranged from 11-4816 cells/cm3, malignant cells were identified during all episodes with same morphological and cytochemical characteristics of identified type of leukaemia. It has been established that the most valuable diagnostic procedure in CNS leukemia is CSF examination, and detection of blasts is sufficient for diagnosis. All other procedures like EEG, myelography and CT have only supplemental diagnostic significance. Finally, in this study we showed that neurological symptomatology in patients with acute leukemia is not dependent of the type of leukemia, moreover different types of AL can have same neurological manifestations. As others, we sometimes used the term CNS leukemia in this paper, although it is clear that meninges and peripheral nervous system are most often involved. This is the reason why we suggest that neuroleukemia, or NS leukemia should be used as more appropriate expressions.

摘要

中枢神经系统受白血病细胞累及是儿童急性淋巴细胞白血病(ALL)的一种公认并发症,但随着全身治疗的近期改善和生存期延长,该并发症在成人中的发生率有所增加。中枢神经系统白血病患者的神经症状是由于脑膜浸润,但有时也由于弥漫性和结节性脑浸润。1991年1月至1994年12月期间,36例急性白血病患者(28例ALL,8例急性髓细胞白血病[AML])经以下标准证实患有神经白血病:(1)腰椎穿刺(LP)时脑脊液中出现细胞增多和原始细胞(细菌学和真菌培养阴性),以及(2)存在神经症状和体征。所有36例患者共发生46次中枢神经系统受累。每次发作时均对所有患者进行神经检查,并根据神经异常情况分为四类。所有患者均进行了LP,通过Sayk室沉淀获得脑脊液沉淀物,常规用MGG和细胞化学染色法检测白血病细胞(图1)。21次发作时进行了脑电图检查,15次进行了CT扫描。我们根据最突出的神经症状和体征将患者分为四组。第一组包括23次发作(50%)(18例ALL,5例AML),其中脑膜刺激症状和体征占主导,类似于脑膜炎的临床表现。当脑脊液检查至关重要时,这种脑膜综合征有时会在与中枢神经系统感染的鉴别诊断中产生问题。第二组为9例患者(6例ALL和3例AML),发作10次(21.74%),其中以颅神经症状和体征为主或仅存在颅神经症状和体征。最常受累的是动眼神经、面神经和视神经。第三组由8例ALL患者(8次发作,17.39%)组成,以脊髓神经根症状为主,由脊髓神经根或其周围脑膜的病理浸润引起。该组还包括1例多灶性单神经炎患者和另1例疼痛性多发性神经病患者。该组所有患者直腿抬高时均有疼痛,但我们在此强调,其他组所有患者拉扎列维奇征也为阳性。因此,它可以作为区分中毒性药物性多发性神经病和白血病性多发性神经根神经病的良好鉴别诊断参数。第四组包括5例患者(5次发作,10.87%)。4例ALL和1例AML,其中以癫痫发作、偏瘫和精神器质性综合征等脑症状为主。所有发作时均通过腰椎穿刺获取脑脊液。蛋白质浓度范围为21 - 3180mg/dl,28次发作时高于正常(45mg/dl)。16次发作时存在轻度脑脊液低糖。细胞计数范围为11 - 4816个细胞/cm³,所有发作时均鉴定出具有所确定白血病类型相同形态学和细胞化学特征的恶性细胞。已确定中枢神经系统白血病最有价值的诊断方法是脑脊液检查,检测到原始细胞足以确诊。脑电图、脊髓造影和CT等所有其他检查仅具有辅助诊断意义。最后,在本研究中我们表明急性白血病患者的神经症状与白血病类型无关,此外不同类型的急性白血病可具有相同的神经表现。与其他人一样,我们在本文中有时使用中枢神经系统白血病这一术语,尽管很明显脑膜和周围神经系统最常受累。这就是为什么我们建议使用神经白血病或NS白血病作为更合适的表述。

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