Thrombosis in individuals with high oxygen affinity hemoglobin‐associated erythrocytosis: A systematic review and analysis of a national cohort
Abstract
High-oxygen-affinity hemoglobins (HOAH) are rare conditions, yet one of the most prevalent causes of constitutional erythrocytosis (CE). 1 In some individuals, the impairment of oxygen delivery to tissues leads to secondary erythrocytosis, 2 although most are asymptomatic.3 Nonetheless, thrombosis and hyperviscosity-related symptoms can occur with an unknown incidence.As most data derive from individual and small case series, there is no consensus on optimal therapeutic management, and it is unclear whether antithrombotic prophylaxis and phlebotomies to reduce hematocrit can be useful, as it has been shown in patients with polycythemia vera (PV).[4][5][6][7] To better understand variables associated with thrombosis and hyperviscosity-related symptoms in individuals with HOAH, we conducted a systematic review of the literature and analyzed a retrospective multicentric cohort as validation.To conduct this review, we led a systematic search of the PubMed database with terms "hemoglobin," "variant," "high affinity," "oxygen," and "erythrocytosis."A PubMed search with all HOAH variants described in the HbVar database was also conducted.Two reviewers (M.L. and C.O.) independently screened all identified studies.The second part of the study focused on individuals with HOAH variants at 16 French institutions.All individuals were informed, and non-opposition to data collection was obtained.The study was approved by an institutional Research Ethics board (Angers University Hospital, #2022-146).Data collection and statistical analyses are described in Supplementary Methods.The systematic search identified 780 publications, of which 552 were assessed for eligibility after duplicate removal.After review, 319 publications not fulfilling inclusion criteria were excluded (Supporting Information S1: Figure S1).For this analysis, we ultimately included 233 publications reaching all our inclusion criteria (Supporting Information S1: Table S1), reporting on a total of 665 individuals.Patient characteristics are detailed in Supporting Information S1: Table S2.At diagnosis, 47 thrombotic events were observed in 37 patients (6.8%) with available clinical data (N = 548), including 28 venous and 19 arterial thrombotic events (Supporting Information S1: Table S3).Patients with thrombosis were older (53 vs. 32 years, P < 0.001) andHemaSphere.
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BibTeX
@article{laloi2026thrombosis,
title = {{Thrombosis in individuals with high oxygen affinity hemoglobin‐associated erythrocytosis: A systematic review and analysis of a national cohort}},
author = {Marie Laloi and Nada Maaziz and Victor Bobée and Philippe Joly and Muriel Giansily-Blaizot and Mathieu Carpentier and Marie‐pierre Gourin and Sébastien Trouillier and Claire Barro and Charles Dumontet and Clémence Santana and Matthieu Wemeau and Mathilde Hunault and Damien Luque-Paz and Serge Pissard and Frédéric Galactéros and François Girodon and Patricia Aguilar-Martinez and Loïc Garcon and Corentin Orvain},
journal = {HemaSphere},
year = {2026},
month = sep,
doi = {10.1002/hem3.70469},
url = {https://u-picardie.hal.science/hal-05753490v1},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Thrombosis in individuals with high oxygen affinity hemoglobin‐associated erythrocytosis: A systematic review and analysis of a national cohort
AU - Marie Laloi
AU - Nada Maaziz
AU - Victor Bobée
AU - Philippe Joly
AU - Muriel Giansily-Blaizot
AU - Mathieu Carpentier
AU - Marie‐pierre Gourin
AU - Sébastien Trouillier
AU - Claire Barro
AU - Charles Dumontet
AU - Clémence Santana
AU - Matthieu Wemeau
AU - Mathilde Hunault
AU - Damien Luque-Paz
AU - Serge Pissard
AU - Frédéric Galactéros
AU - François Girodon
AU - Patricia Aguilar-Martinez
AU - Loïc Garcon
AU - Corentin Orvain
PY - 2026
DA - 2026/09/09
JO - HemaSphere
DO - 10.1002/hem3.70469
UR - https://u-picardie.hal.science/hal-05753490v1
AB - High-oxygen-affinity hemoglobins (HOAH) are rare conditions, yet one of the most prevalent causes of constitutional erythrocytosis (CE). 1 In some individuals, the impairment of oxygen delivery to tissues leads to secondary erythrocytosis, 2 although most are asymptomatic.3 Nonetheless, thrombosis and hyperviscosity-related symptoms can occur with an unknown incidence.As most data derive from individual and small case series, there is no consensus on optimal therapeutic management, and it is unclear whether antithrombotic prophylaxis and phlebotomies to reduce hematocrit can be useful, as it has been shown in patients with polycythemia vera (PV).[4][5][6][7] To better understand variables associated with thrombosis and hyperviscosity-related symptoms in individuals with HOAH, we conducted a systematic review of the literature and analyzed a retrospective multicentric cohort as validation.To conduct this review, we led a systematic search of the PubMed database with terms "hemoglobin," "variant," "high affinity," "oxygen," and "erythrocytosis."A PubMed search with all HOAH variants described in the HbVar database was also conducted.Two reviewers (M.L. and C.O.) independently screened all identified studies.The second part of the study focused on individuals with HOAH variants at 16 French institutions.All individuals were informed, and non-opposition to data collection was obtained.The study was approved by an institutional Research Ethics board (Angers University Hospital, #2022-146).Data collection and statistical analyses are described in Supplementary Methods.The systematic search identified 780 publications, of which 552 were assessed for eligibility after duplicate removal.After review, 319 publications not fulfilling inclusion criteria were excluded (Supporting Information S1: Figure S1).For this analysis, we ultimately included 233 publications reaching all our inclusion criteria (Supporting Information S1: Table S1), reporting on a total of 665 individuals.Patient characteristics are detailed in Supporting Information S1: Table S2.At diagnosis, 47 thrombotic events were observed in 37 patients (6.8%) with available clinical data (N = 548), including 28 venous and 19 arterial thrombotic events (Supporting Information S1: Table S3).Patients with thrombosis were older (53 vs. 32 years, P < 0.001) andHemaSphere.
SN - 2572-9241
ER -