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4号染色体三体:临床表现、血液学及生存情况。两例病例报告并文献复习。

Trisomy 4: clinical picture, hematology, and survival. Presentation of two cases and review of the literature.

作者信息

Pedersen B

机构信息

Danish Cancer Society, Department of Cytogenetics, Aarhus, Amtssygehus.

出版信息

Hematol Pathol. 1992;6(3):161-7.

PMID:1429344
Abstract

Two patients with acute nonlymphocytic leukemia (ANLL) and trisomy 4 as the only cytogenetic aberration are presented. Including the two, a total of 31 cases with this karyotype have been described till now. A review of the 31 cases shows that they fall into two distinct age groups, a younger group of 10 patients (5-34 years) and an older group of 21 patients (50-75 years). With four exceptions the diagnosis was ANLL in all cases, mostly FAB classes M2 and M4. At the time of diagnosis 18 had clinical signs of active infection. In 20 cases the peripheral leukocyte count was above 10 x 10(9)/L but not associated with presence/absence of infection. The median survival was 17 months from diagnosis. Independent prognostic factors were diagnosis (p = 0.01), peripheral leukocyte count (p = 0.03), and percentage of metaphases with trisomy 4 (p = 0.04). The two age groups as well as presence/absence of infection at the time of diagnosis were without significant prognostic consequences.

摘要

本文报告了2例急性非淋巴细胞白血病(ANLL)患者,其唯一的细胞遗传学异常为4号染色体三体。包括这2例患者在内,目前共报道了31例具有这种核型的病例。对这31例病例的回顾显示,它们分为两个不同的年龄组,一个较年轻的组有10例患者(5 - 34岁),一个较年长的组有21例患者(50 - 75岁)。除4例例外情况外,所有病例的诊断均为ANLL,大多数为FAB M2和M4型。诊断时18例有活动性感染的临床体征。20例患者外周血白细胞计数高于10×10⁹/L,但与有无感染无关。自诊断起的中位生存期为17个月。独立的预后因素为诊断(p = 0.01)、外周血白细胞计数(p = 0.03)以及4号染色体三体的中期细胞百分比(p = 0.04)。两个年龄组以及诊断时有无感染均无显著的预后影响。

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