Current hematological findings in cobalamin deficiency.: A study of 201 consecutive patients with documented cobalamin deficiency
Current hematological findings in cobalamin deficiency.: A study of 201 consecutive patients with documented cobalamin deficiency
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DOI：
10.1111/j.1365-2257.2006.00755.x
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发表时间：
2006-02-01
期刊：
影响因子：
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通讯作者：
Blicklé, JF
中科院分区：
文献类型：
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作者：
Andrès, E;Affenberger, S;Blicklé, JF
在过去的几十年中，随着用于测量血清钴胺素水平的自动化测定的引入，与钴胺素缺乏相关的血液学表现已经从“旧”研究或教科书中报道的描述发生了变化。我们研究了从一项观察性队列研究（1995-2003年）中提取的201例（中位年龄：67 +/- 6岁）有充分记录的钴胺素缺乏症（平均血清维生素B12水平125 +/- 47 pg/ml）患者的血液学表现或异常。评估包括临床特征、血细胞计数和形态学检查。至少三分之二的患者报告了血液学异常：贫血（37%）、白细胞减少症（13.9%）、血小板减少症（9.9%）、巨红细胞增多症（54%）和中性粒细胞增多症（32%）。平均血红蛋白水平为10.3 +/- 0.4 g/dl，平均红细胞体积为98.9 +/- 25.6 fl。约10%的患者有危及生命的血液学表现，有记录的症状性全血细胞减少症（5%）、“假性”血栓性微血管病（Moschkowitz; 2.5%）、重度贫血（定义为Hb水平< 6 g/dl; 2.5%）和溶血性贫血（1.5%）。至少有三分之二的患者血液学异常得到纠正，肌肉注射或口服结晶氰钴胺治疗的患者同样如此。这项研究，基于真实的数据，从一个单一的机构与大量的连续患者有充分的证明钴胺素缺乏症，证实了几个“老”的发现，以前报告的几个研究和教科书在20世纪90年代之前。
With the introduction of automated assays for measuring serum cobalamin levels over the last decades, the hematological manifestations related to cobalamin deficiency have been changed from the description reported in 'old' studies or textbooks. We studied the hematological manifestations or abnormalities in 201 patients (median age: 67 +/- 6 years) with well-documented cobalamin deficiency (mean serum vitamin B12 levels 125 +/- 47 pg/ml) extracted from an observational cohort study (1995-2003). Assessment included clinical features, blood count and morphological review. Hematological abnormalities were reported in at least two-third of the patients: anemia (37%), leukopenia (13.9%), thrombopenia (9.9%), macrocytosis (54%) and hypegmented neutrophils (32%). The mean hemoglobin level was 10.3 +/- 0.4 g/dl and the mean erythrocyte cell volume 98.9 +/- 25.6 fl. Approximately 10% of the patients have life-threatening hematological manifestations with documented symptomatic pancytopenia (5%), 'pseudo' thrombotic microangiopathy (Moschkowitz; 2.5%), severe anemia (defined as Hb levels < 6 g/dl; 2.5%) and hemolytic anemia (1.5%). Correction of the hematological abnormalities was achieved in at least two-thirds of the patients, equally well in patients treated with either intramuscular or oral crystalline cyanocobalamin. This study, based on real data from a single institution with a large number of consecutive patients with well-documented cobalamin deficiency, confirms several 'older' findings that were previously reported before the 1990s in several studies and in textbooks.