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

Autor: Drouilly M; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Jourdan L; Service de Néonatologie, CHRU Nancy, Nancy, France., Gérard D; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Russello J; Service d'Hématologie Biologique, CHU Montpellier, Montpellier, France., Bobée V; Service d'Hématologie Biologique, CHU Rouen, Rouen, France., Audouy A; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France., Phulpin A; Service d'Onco-Hématologie Pédiatrique, CHRU Nancy, Nancy, France., Perrin J; Service d'Hématologie Biologique, CHRU Nancy, Nancy, France.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2024 Aug; Vol. 71 (8), pp. e31078. Date of Electronic Publication: 2024 May 24.
DOI: 10.1002/pbc.31078
Abstrakt: 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.)
Databáze: MEDLINE
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