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[在一家老年医院诊断出的骨髓增生异常综合征:100例患者的形态学特征]

[Myelodisplasic syndromes diagnosed in a geriatric hospital: morphological profile in 100 patients].

作者信息

Dewulf G, Gouin I, Pautas E, Gaussem P, Chaïbi P, Andreux J-P, Siguret V

机构信息

Laboratoire d'hématologie, Hôpital Charles Foix (AP-HP), Ivry-sur-Seine.

出版信息

Ann Biol Clin (Paris). 2004 Mar-Apr;62(2):197-202.

Abstract

Myelodysplastic syndrome (MDS) is particularly common in geriatric practice. As few data are available in very elderly patients, we conducted a 54-month retrospective study in patients over 70 years with MDS diagnosed at Hôpital Charles Foix. Patients with cobalamine, folate or iron deficiency were excluded. Regarding biological and morphologic approaches, MDS patients were classified according to the FAB criteria. We then tempted to reclassify the patients according to the WHO criteria. The Bournemouth scoring system was used as a prognostic tool. During the study period, 100 patients were included, 29 males and 71 females, median age 86 +/- 7 years (70-103). At the time of bone marrow sampling, a peripheral blood cytopenia was documented in 64 patients, a bicytopenia in 27 patients and a pancytopenia in 9 patients. Isolated anaemia (Hb < 12 g/dL) was found in 60 patients and isolated thrombocytopenia (< 150 x 10(9)/L) in 4. Macrocytosis (MCV > 100 fL) was observed in 21 % of the cases. According to the FAB criteria, the 100 patients were classified as follows: refractory anaemia (RA): 79%; RA with ringed sideroblasts (RARS): 8%; RA with excess of blasts (RAEB): 8%; RAEB in transformation: 1%; chronic myelomonocytic leukaemia: 4%. According to the WHO classification, the patients were reclassified as follows: RA (unilineage) (with or without ringed sideroblasts): 10%; refractory cytopenia with multilineage dysplasia with or without ringed sideroblasts (RCMD): 73%; RAEB: 7% (RAEB-1 6%, RAEB-21%); MDS/Myeloproliferative disorder: 4%; unclassified (hypocellularity): 5%; acute leukaemia: 1%. In order to estimate prognosis at the time of the bone marrow aspirate, we calculated the Bournemouth'score: 8 patients scored 0,57 scored 1,25 scored 2,8 scored 3 and 2 scored 4. In this geriatric population, 83% cases of MDS are RA or RCMD (with or without sideroblasts); MDS with excess of blasts are uncommon. Thus, elderly patients under study with MDS were diagnosed at an earlier stage of the disease than younger ones from series published in the literature. Due to frequent comorbidities, geriatric patients may be symptomatic for a slight decrease of haemoglobin level. Therefore, elderly patients are investigated as soon as they present with moderate anaemia that may explain the early MDS diagnosis.

摘要

骨髓增生异常综合征(MDS)在老年医学实践中尤为常见。由于关于高龄患者的数据较少,我们对在查尔斯·福瓦医院确诊为MDS的70岁以上患者进行了一项为期54个月的回顾性研究。排除了钴胺素、叶酸或铁缺乏的患者。关于生物学和形态学方法,MDS患者根据FAB标准进行分类。然后我们尝试根据世界卫生组织(WHO)标准对患者重新分类。使用伯恩茅斯评分系统作为预后工具。在研究期间,纳入了100例患者,其中男性29例,女性71例,中位年龄86±7岁(70 - 103岁)。在骨髓采样时,64例患者记录有外周血细胞减少,27例患者有双系血细胞减少,9例患者有全血细胞减少。60例患者发现单纯贫血(血红蛋白<12 g/dL),4例患者发现单纯血小板减少(<150×10⁹/L)。21%的病例观察到巨细胞性(平均红细胞体积>100 fL)。根据FAB标准,100例患者分类如下:难治性贫血(RA):79%;环形铁粒幼细胞性难治性贫血(RARS):8%;伴原始细胞增多的难治性贫血(RAEB):8%;转化中的RAEB:1%;慢性粒单核细胞白血病:4%。根据WHO分类,患者重新分类如下:RA(单系)(有或无环形铁粒幼细胞):10%;伴有或不伴有环形铁粒幼细胞的多系发育异常的难治性血细胞减少(RCMD):73%;RAEB:7%(RAEB - 1 6%,RAEB - 2 1%);MDS/骨髓增殖性疾病:4%;未分类(细胞减少):5%;急性白血病:1%。为了在骨髓穿刺时评估预后,我们计算了伯恩茅斯评分:8例患者得0分,57例得1分,25例得2分,8例得3分,2例得4分。在这个老年人群中,83%的MDS病例为RA或RCMD(有或无铁粒幼细胞);伴原始细胞增多的MDS不常见。因此,本研究中的老年MDS患者比文献中报道的年轻患者在疾病的更早阶段被诊断出来。由于合并症频繁,老年患者可能因血红蛋白水平轻微下降而出现症状。因此,老年患者一旦出现中度贫血就会接受检查,这可能解释了MDS的早期诊断。

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