Hematological abnormalities during the first week of life among neonates with Down syndrome: Data from a multihospital healthcare system
Hematological abnormalities during the first week of life among neonates with Down syndrome: Data from a multihospital healthcare system
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DOI：
10.1002/ajmg.a.31442
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发表时间：
2007-01-01
影响因子：
2
通讯作者：
Christensen, R. D.
中科院分区：
文献类型：
--
作者：
Henry, E.;Walker, D.;Christensen, R. D.
据报道，患有唐氏综合症的新生儿存在各种血液学异常。血小板增多症、血小板减少症、红细胞增多症、中性粒细胞增多症、短暂性骨髓增殖性疾病（TMD）和先天性白血病都有报道。此前报道的两个最大的病例系列涉及 63 例和 31 例。为了从更大的病例系列中获取血液学数据，我们获得了 Intermountain Healthcare QHQ 医院治疗的所有唐氏综合症新生儿出生后第一周内完成的所有 CBC，出生日期为 2001 年 1 月 1 日至 2005 年 12 月 31 日。在此期间，18 家医院记录了 145,522 例活产。 226 人（644 人中有 1 人）被诊断出患有唐氏综合症。其中一百五十八 (70%) 在第七次粘土（144 小时）之前获得了一个或多个 CBC。第一周内进行 CBC 检查的新生儿与未进行 CBC 检查的新生儿具有相似的胎龄、LGA 和 SGA 出生体重百分比以及住院时间。中性粒细胞增多症是最常见的血液学异常，80% 的中性粒细胞绝对计数高于年龄正常上限。百分之六 (9/158) 在血涂片上发现了原始细胞，其中三个（如果这种情况持续存在）被转至儿科血液学服务机构进行进一步评估。其次最常检测到的异常是血小板减少症，66% 的血小板计数 < 150,000/μl，6% 的 coLints < 50,000/μl。平均血小板体积与血小板计数无关，但往往稍大（9.2 +/- 1.3 fl），其中 24% 的值高于 10 fl。只有一名患者接受了血小板输注。红细胞增多症是第二常见的血液异常，33% 的红细胞压积值高于 65% 或血红蛋白浓度高于 22 g/dl。六人接受减量输血。一名患者患有严重贫血（血细胞比容<15%）并接受了红细胞输注。一名患者出现与肠道手术后感染相关的中性粒细胞减少症。中性粒细胞增多症、血小板减少症和红细胞增多症是唐氏综合症新生儿中最常见的血液学异常。贫血、血小板增多症和中性粒细胞减少症并不比没有唐氏综合症的新生儿更常见。血液学异常在该组中非常常见，因此建议他对所有患有唐氏综合症的新生儿进行一项或多项全血细胞计数似乎是合理的。 (c) 2006 Wiley-Liss, Inc.
Various hematological abnormalities have been reported among neonates with Down syndrome. Thrombocytosis, thrombocytopenia, polycythemia, neutrophilia, transient myeloproliferative disorder (TMD), and congenital leukemia have all been reported. The two largest case series previously reported involved 63 and 31 cases. To acquire hematological data from a larger case series, we obtained all CBCs done during the first week after birth on all neonates with Down syndrome cared for in an Intermountain Healthcare QHQ hospital with a date of birth between January 1, 2001 and December 31, 2005. During this period, 145,522 live births were recorded at 18 hospitals. Down syndrome was recognized in 226 (1 in 644). One hundred fifty-eight (70%) of these had one or more CBCs obtained before the seventh clay (144 hr). Neonates who did versus did not have a CBC in the first week had a similar gestational age, birth weight percentage who were LGA and SGA, and length of stay. Neutrophilia was the most common hematological abnormality detected, with 80% of absolute neutrophil counts above the Upper limit of normal for age. Six percent (9/158) had blasts identified on the blood film and three, where this was persistent, were referred to the pediatric hematology service for further evaluation. The next most commonly detected abnormality was thrombocytopenia, with 66% of platelet counts < 150,000/mu l, and with 6% of coLints < 50,000/mu l. The mean platelet volume did not correlate with the platelet count, but tended to run slightly large (9.2 +/- 1.3 fl), with 24% of values above 10 fl. Only one had a platelet transfusion. Polycythemia was the next most common hematological abnomality detected, with 33% of hematocrit values above 65% or hemoglobin concentations ahove 22 g/dl. Six had a reduction transfusion. One patient had significant anemia (hematocrit < 15%) and received an erythrocyte transfusion. One had neutropenia associated with an infection after bowel surgery. Nentrophilia, thrombocytopenia, and polvcythemia were the most common hematological abnormalities observed among neonates with Down syndrome. Anemia, thrombocytosis, and neutropenia were not more common than among, neonates who do not have Down syndrome. Hematological abnormalities were so common in this group that it seems reasonable to recommend that one or more CBCs he obtained on all neonates with Down syndrome. (c) 2006 Wiley-Liss, Inc.