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Erythrocytosis associated with IgA nephropathy

Erythrocytosis is a hematological disorder usually related to hematopoietic stem cell somatic mutations. However, unexplained erythrocytosis remains frequent. In this study, we evaluated the involvement of IgA1, a regulator of erythropoiesis also implicated in IgA nephropathy (IgAN) pathophysiology,...

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Published in:EBioMedicine 2022-01, Vol.75, p.103785-103785, Article 103785
Main Authors: Cohen, Camille, Coulon, Séverine, Bhukhai, Kanit, Neuraz, Antoine, Dussiot, Michael, Fouquet, Guillemette, Stang, Marie-Bénédicte Le, Flamant, Martin, Vrtovsnik, François, Hummel, Aurélie, Knebelmann, Bertrand, Mesnard, Laurent, Rondeau, Eric, Maciel, Thiago T., Favale, Fabrizia, Casadevall, Nicole, Nguyen-Khoa, Thao, Moutereau, Stéphane, Legendre, Christophe, Benhamou, Marc, Monteiro, Renato C., Hermine, Olivier, El Karoui, Khalil, Moura, Ivan C.
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Language:English
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Summary:Erythrocytosis is a hematological disorder usually related to hematopoietic stem cell somatic mutations. However, unexplained erythrocytosis remains frequent. In this study, we evaluated the involvement of IgA1, a regulator of erythropoiesis also implicated in IgA nephropathy (IgAN) pathophysiology, in unexplained polycythemia/erythrocytosis (PE) of IgAN patients. IgAN-PE patients’ serum was collected, analyzed and used to study IgA1 effect on proliferation and differentiation of erythroid progenitors. Hematological parameters of transgenic mice for human alpha1 heavy chain were studied. Multicentric observational cohorts of chronic kidney disease (CKD) patients, including both native kidney diseases and renal transplants, were studied to analyze patient hemoglobin levels. We retrospectively identified 6 patients with IgAN and unexplained PE. In large CKD cohorts, IgAN was associated with PE in 3.5% of patients (p
ISSN:2352-3964
2352-3964
DOI:10.1016/j.ebiom.2021.103785