与嵌合型唐氏综合征相关的急性巨核细胞白血病。

Acute megakaryoblastic leukemia associated with mosaic Down's syndrome.

作者信息

Simon J H, Tebbi C K, Freeman A I, Brecher M L, Green D M, Sandberg A A

机构信息

Department of Pediatrics, Roswell Park Memorial Institute, Buffalo, NY 14263.

出版信息

Cancer. 1987 Nov 15;60(10):2515-20. doi: 10.1002/1097-0142(19871115)60:10<2515::aid-cncr2820601028>3.0.co;2-f.

DOI:10.1002/1097-0142(19871115)60:10<2515::aid-cncr2820601028>3.0.co;2-f

PMID:2959356

Abstract

There have been several reports of the association between Down's Syndrome and acute megakaryoblastic (M7) leukemia (AMKL). The diagnosis of this rare form of leukemia has been better delineated by the use of the platelet peroxidase reaction and the antifactor VIII antibody immunoperoxidase test. In the past, the prognosis of patients with a combination of Down's Syndrome and AMKL has been extremely poor, with a median survival of 6.9 months for 11 reported cases in the literature. The present report reviews the previously reported cases and describes a unique patient with mosaic Down's syndrome and AMKL with favorable response to therapy.

摘要

已有多篇关于唐氏综合征与急性巨核细胞白血病（M7型，AMKL）关联的报道。血小板过氧化物酶反应和抗因子VIII抗体免疫过氧化物酶检测的应用，使这种罕见白血病的诊断得到了更好的界定。过去，唐氏综合征合并AMKL患者的预后极差，文献报道的11例患者中位生存期为6.9个月。本报告回顾了先前报道的病例，并描述了一名患有嵌合型唐氏综合征和AMKL且对治疗反应良好的独特患者。