研究动态
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婴儿固缩细胞增多症,一种新生儿亨氏小体溶血性贫血:一项队列研究。

Infantile pyknocytosis, a neonatal hemolytic anemia with Heinz bodies: A cohort study.

发表日期:2024 May 24
作者: Marie Drouilly, Laurélia Jourdan, Delphine Gérard, Jennifer Russello, Victor Bobée, Alexia Audouy, Aurélie Phulpin, Julien Perrin
来源: Immunity & Ageing

摘要:

婴儿固缩红细胞增多症 (IP) 是一种罕见的、可能被错误估计的非免疫性新生儿溶血性贫血病因,分两个阶段发展:初始阶段出现严重黄疸,随后出现第二阶段溶血性贫血,可能需要新生儿重症监护。 IP 的诊断基于血涂片上短暂存在的高密度、收缩和/或有刺的红细胞(密核细胞),与临床生物学特征的自发消退和排除其他原因相关。如果病因仍未确定,则有人提出了一些影响因素,例如氧化应激。我们报告了 16 名 IP 患者的描述,旨在澄清与这种获得性疾病发展相关的情况。在急性期,平均血红蛋白最低点和致密细胞计数分别为 7.8 g/dL 和 11%,引人注目的是,亨氏小体在 50% 的新生儿中很明显,但在长时间潜伏期(4 小时)后这一比例为 100%。新生儿中地中海或非洲血统的比例很高,并且有大量围产期事件,例如呼吸窘迫。如果 IP 的病因肯定是多因素的,那么我们的系列文章强调了氧化应激的作用,氧化应激可能至少部分起源于新生儿的去饱和事件。© 2024 作者。儿科血液
Infantile pyknocytosis (IP) is a rare, probably misestimated, cause of non-immune neonatal hemolytic anemia evolving in two phases: an initial phase with severe jaundice, followed by a second phase with hemolytic anemia, which may require neonatal intensive care. The diagnosis of IP is based on the transient presence on blood smear of hyperdense, contracted, and/or spiculated red blood cells (pyknocytes), associated with the spontaneous resolution of clinico-biological features and the exclusion of other causes. If the etiology remains undetermined, some contributing factors, such as oxidative stress, have been proposed. We report the description of 16 patients with IP aiming at clarifying the circumstances associated with the development of this acquired disorder. In the acute phase, the mean hemoglobin nadir and pyknocyte count were 7.8 g/dL and 11%, respectively, and strikingly, Heinz bodies were evident in 50% of the newborns, but in 100% after prolonged incubation (4 hours). A high proportion of Mediterranean or African ancestry was noted in newborns, as well as a significant number of peripartum events, such as respiratory distress. If the etiology of IP is certainly multifactorial, our series reinforces the role of oxidative stress, which may, at least in part, find origin in desaturation episodes in newborns.© 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.