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伴有骨髓嗜酸性粒细胞增多的骨髓增生异常综合征:临床及细胞遗传学特征

Myelodysplastic syndrome with bone marrow eosinophilia: clinical and cytogenetic features.

作者信息

Matsushima T, Murakami H, Tsuchiya J

机构信息

Third Department of Internal Medicine, Gunma University School of Medicine, Japan.

出版信息

Leuk Lymphoma. 1994 Nov;15(5-6):491-7. doi: 10.3109/10428199409049753.

Abstract

We investigated the hematological and clinical status of 145 patients with de novo myelodysplastic syndrome (MDS), 14 of whom (10%) had eosinophilia in the bone marrow (MDS-Eo). Most of these 14 patients had severe anemia. Their bone marrow cells exhibited trilineage dysplasia and some morphological abnormalities in the eosinophils, including disproportion of eosinophilic granules, basophilic granules, a ring-shaped nucleus, and vacuolation in the cytoplasm. However, these abnormalities were less prominent than those of acute myelomonocytic leukemia with eosinophilia (FAB: M4Eo). Three of the 14 MDS-Eo patients had refractory anemia (RA), seven had RA with excess of blasts (RAEB), and four had RAEB in transformation. Cytogenetic analysis revealed chromosomal abnormalities in 12 of 13 MDS-Eo patients (92%), in particular, there were major karyotypic abnormalities (MAKA) in eight patients (62%). Cytotoxic agents were not effective in the treatment of four patients after leukemic transformation occurred. These four patients died of the leukemic transformation while seven died of bone marrow failure. The other three MDS-Eo patients are still alive; two of them have already transformed to a leukemic phase. The duration of survival of these patients was significantly shorter than that of the other MDS patients. These findings suggest that bone marrow eosinophilia in MDS may be a poor prognostic factor that is strongly related to the existence of MAKA.

摘要

我们调查了145例初诊骨髓增生异常综合征(MDS)患者的血液学和临床状况,其中14例(10%)骨髓中存在嗜酸性粒细胞增多(MDS-Eo)。这14例患者大多有严重贫血。他们的骨髓细胞表现出三系发育异常,嗜酸性粒细胞存在一些形态学异常,包括嗜酸性颗粒、嗜碱性颗粒比例失调、核呈环状以及细胞质有空泡形成。然而,这些异常不如伴有嗜酸性粒细胞增多的急性粒单核细胞白血病(FAB:M4Eo)明显。14例MDS-Eo患者中,3例为难治性贫血(RA),7例为伴有过多原始细胞的难治性贫血(RAEB),4例为转化中的RAEB。细胞遗传学分析显示,13例MDS-Eo患者中有12例(92%)存在染色体异常,特别是8例患者(62%)有主要核型异常(MAKA)。4例患者发生白血病转化后,细胞毒性药物治疗无效。这4例患者死于白血病转化,7例死于骨髓衰竭。另外3例MDS-Eo患者仍存活;其中2例已转化为白血病期。这些患者的生存期明显短于其他MDS患者。这些发现表明,MDS中的骨髓嗜酸性粒细胞增多可能是一个不良预后因素,与MAKA的存在密切相关。

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