Paulus J A, Bos G M, Löwenberg B, Van Den Bent M J
Academisch Ziekenhuis Rotterdam-Daniel den Hoed Kliniek, Rotterdam.
Ned Tijdschr Geneeskd. 1998 Oct 3;142(40):2196-200.
A description of clinical features and treatment results in patients with a central nervous system (CNS) localization of systemic non-Hodgkin's lymphoma (NHL).
Retrospective.
Department of Neuro-oncology and Haematology, Daniel den Hoed Cancer Centre, Rotterdam, the Netherlands.
All patients with NHL (but not primary CNS lymphoma) treated in the period January 1st, 1990-December 31st, 1996 at the department of neuro-oncology were reviewed for presence of CNS localizations. Clinical characteristics like malignancy grade, disease stage, presence of extranodal localizations, B-symptoms, serum LDH, neurological signs and symptoms, results of ancillary investigations, treatment and response were recorded.
In this period 25 patients were diagnosed with leptomeningeal lymphoma, 2 with an intracerebral lymphoma, and 7 with both. In almost all patients the CNS localization developed during systemic progression of the NHL. Most patients presented with a radicular syndrome, cranial nerve deficits, headache or encephalopathy. More than 80% of the patients showed clinical improvement after treatment with intrathecal chemotherapy, radiation therapy or a combination of both. The median survival was three and a half months, six month survival was 32%. Progression of systemic disease was the most frequent cause of death.
In most patients good palliation of neurological signs and symptoms could be obtained with intrathecal chemotherapy and radiation therapy. Survival in these patients was limited, however, in part because of the frequent concurrent progression of systemic disease. Patients with CNS localizations with chemotherapeutic treatment possibilities with a realistic chance of cure or longterm survival should be treated accordingly. Otherwise, treatment should consist of intrathecal chemotherapy or radiation therapy of involved areas only.
描述系统性非霍奇金淋巴瘤(NHL)中枢神经系统(CNS)定位患者的临床特征及治疗结果。
回顾性研究。
荷兰鹿特丹丹尼尔·登霍德癌症中心神经肿瘤学与血液学系。
对1990年1月1日至1996年12月31日期间在神经肿瘤学系接受治疗的所有NHL患者(但不包括原发性中枢神经系统淋巴瘤)进行回顾,以确定是否存在CNS定位。记录恶性程度、疾病分期、结外定位情况、B症状、血清乳酸脱氢酶、神经体征和症状、辅助检查结果、治疗及反应等临床特征。
在此期间,25例患者被诊断为软脑膜淋巴瘤,2例为脑内淋巴瘤,7例两者均有。几乎所有患者的CNS定位均在NHL全身进展期间出现。大多数患者表现为神经根综合征、颅神经缺损、头痛或脑病。超过80%的患者在接受鞘内化疗、放射治疗或两者联合治疗后临床症状改善。中位生存期为三个半月,6个月生存率为32%。全身疾病进展是最常见的死亡原因。
对于大多数患者,鞘内化疗和放射治疗可有效缓解神经体征和症状。然而,这些患者的生存期有限,部分原因是全身疾病常同时进展。对于有化疗治愈或长期生存实际机会的CNS定位患者,应相应进行治疗。否则,治疗应仅包括鞘内化疗或对受累区域进行放射治疗。