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慢性粒细胞白血病急变期的脑膜白血病

Meningeal leukemia in the blastic phase of chronic granulocytic leukemia.

作者信息

Schwartz J H, Canellos G P, Young R C, DeVita V T

出版信息

Am J Med. 1975 Dec;59(6):819-28. doi: 10.1016/0002-9343(75)90467-2.

Abstract

One hundred one patients were treated for Ph' positive chronic granulocytic leukemia (CGL) in the blastic phase. In seven of these (6.9 per cent), meningeal leukemia developed. Of the 99 patients who died of their disease, a complete remission was achieved in 12 with a median survival of 12 months (three to 28 months). Incomplete responders had a median survival of only 2.5 months (one to 14 months). In five of the 12 complete responders (42 per cent), but in only two of the incomplete responders (2.3 per cent), meningeal leukemia developed. The principal neurologic signs were cranial nerve palsies and papilledema. All patients had pleocytosis with myeloblasts in the cerebrospinal fluid. As in patients with acute leukemia and diffuse histiocytic lymphoma, increased survival of patients in whom hematologic remission from the blastic phase of CGL is achieved may allow sufficient time for the development of meningeal leukemia. Intrathecal methotrexate is extremely successful in treating this complication. Cerebrospinal fluid pleocytosis was eradicated in all seven of our patients, and neurologic symptoms and signs were completely eliminated in five patients. No evidence of meningeal leukemia was found in three of the five patients in whom an autopsy was performed.

摘要

101例处于急变期的Ph'阳性慢性粒细胞白血病(CGL)患者接受了治疗。其中7例(6.9%)发生了脑膜白血病。在99例死于该病的患者中,12例实现了完全缓解,中位生存期为12个月(3至28个月)。未完全缓解者的中位生存期仅为2.5个月(1至14个月)。12例完全缓解者中有5例(42%)发生了脑膜白血病,但未完全缓解者中只有2例(2.3%)发生。主要神经体征为颅神经麻痹和视乳头水肿。所有患者脑脊液中均有髓母细胞增多。与急性白血病和弥漫性组织细胞淋巴瘤患者一样,CGL急变期实现血液学缓解的患者生存期延长,可能会有足够时间发生脑膜白血病。鞘内注射甲氨蝶呤治疗这种并发症极为成功。我们的7例患者脑脊液中的细胞增多均被消除,5例患者的神经症状和体征完全消失。在接受尸检的5例患者中,有3例未发现脑膜白血病的证据。

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