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唐氏综合征新生儿伴有巨核细胞特征的类白血病反应。

Leukaemoid reaction with megakaryocytic features in newborns with Down's syndrome.

作者信息

Cantù-Rajnoldi A, Cattoretti G, Caccamo M L, Biasini A, Bagnato L, Schirò R, Polli N

机构信息

Laboratorio di Ricerche Cliniche, Istituti Clinici di Perfezionamento, Milano, Italy.

出版信息

Eur J Haematol. 1988 May;40(5):403-9. doi: 10.1111/j.1600-0609.1988.tb00848.x.

Abstract

A leukaemoid reaction was observed in 3 newborns with Down's syndrome. Thrombocytopenia was present in 2, requiring platelets transfusions in 1, and red cell transfusions were necessary in 2 patients. Blast cells characterization by specific monoclonal antibodies showed a prevalence of megakaryoblasts in all 3 cases. This feature was confirmed in 2 of them by the demonstration of platelet peroxidase (PPO) activity under transmission electron microscopy (TEM). A spontaneous remission of the leukaemoid picture was observed after 2-3 months. However, in 1 case a relapse of the myeloproliferative disorder with the same features of the blast cell population was diagnosed after 16 months. Chemotherapy with low-dose Ara-C, started because of a relevant clinical involvement, induced a complete remission.

摘要

在3例唐氏综合征新生儿中观察到类白血病反应。2例存在血小板减少症,其中1例需要输注血小板,2例患者需要输注红细胞。通过特异性单克隆抗体对原始细胞进行鉴定,结果显示所有3例中巨核母细胞均占优势。其中2例通过透射电子显微镜(TEM)显示血小板过氧化物酶(PPO)活性得以证实这一特征。2 - 3个月后观察到类白血病表现自发缓解。然而,1例在16个月后被诊断为具有相同原始细胞群体特征的骨髓增殖性疾病复发。由于临床症状明显而开始使用小剂量阿糖胞苷进行化疗,诱导出现完全缓解。

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