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成人中枢神经系统白血病:发病率及临床病理特征

Adult central nervous system leukemia: incidence and clinicopathologic features.

作者信息

Law I P, Blom J

出版信息

South Med J. 1976 Aug;69(8):1054-7. doi: 10.1097/00007611-197608000-00031.

DOI:10.1097/00007611-197608000-00031
PMID:822517
Abstract

Two hundred and seventy-two adults diagnosed between 1949 and 1971 as having acute leukemia were evaluated. Two hundred and fifty-seven patients had died and autopsies were obtained in 202 cases. Central nervous system (CNS) leukemia was demonstrated in 22 of 93 autopsies with acute nonlymphocytic leukemia (ANLL) during the period 1949 through 1966 and 8 of 47 during the period 1967 through 1971. Nine of 45 autopsies on acute lymphoblastic leukemia (ALL) patients diagnosed during 1949 through 1966 had CNS involvement, compared to 7 of 17 during 1967 through 1971. The median time from diagnosis of acute leukemia to CNS manifestations was two months for ANLL and six months for ALL. Headache, papilledema, and cranial nerve palsy were the common findings with meningeal leukemia. Early CNS involvement was observed in patients with high initial leukocyte/blast counts, low platelet counts, and early lymphadenopathy and hepatosplenomegaly. Ten of 13 patients treated between 1967 and 1971 with cranial irradiation and intrathecal chemotherapy responded; however, the duration of remission in ALL was short-lived with subsequent relapses at various intervals. In contrast, CNS recurrence in ANLL was rare. The value of CNS prophylactic and maintenance therapy is discussed.

摘要

对1949年至1971年间诊断为急性白血病的272名成年人进行了评估。257名患者已死亡,其中202例进行了尸检。在1949年至1966年期间,93例急性非淋巴细胞白血病(ANLL)尸检中有22例显示中枢神经系统(CNS)白血病,1967年至1971年期间47例中有8例。1949年至1966年期间诊断的45例急性淋巴细胞白血病(ALL)患者尸检中有9例出现CNS受累,1967年至1971年期间17例中有7例。ANLL从急性白血病诊断到CNS表现的中位时间为2个月,ALL为6个月。头痛、视乳头水肿和颅神经麻痹是脑膜白血病的常见表现。在初始白细胞/原始细胞计数高、血小板计数低以及早期出现淋巴结病和肝脾肿大的患者中观察到早期CNS受累。1967年至1971年期间接受颅脑照射和鞘内化疗的13例患者中有10例有反应;然而,ALL的缓解期短暂,随后在不同间隔复发。相比之下,ANLL的CNS复发很少见。文中讨论了CNS预防性和维持性治疗的价值。

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