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Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience

Erythrocytosis has a diverse background. While polycythaemia vera has well defined criteria, the diagnostic approach and management of other types of erythrocytosis are more challenging. The aim of study was to retrospectively analyse the aetiology and management of non-clonal erythrocytosis patient...

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Autores principales: Anžej Doma, Saša, Drnovšek, Eva, Kristan, Aleša, Fink, Martina, Sever, Matjaž, Podgornik, Helena, Belčič Mikič, Tanja, Debeljak, Nataša, Preložnik Zupan, Irena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2021
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8285333/
https://www.ncbi.nlm.nih.gov/pubmed/34013406
http://dx.doi.org/10.1007/s00277-021-04546-4
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author Anžej Doma, Saša
Drnovšek, Eva
Kristan, Aleša
Fink, Martina
Sever, Matjaž
Podgornik, Helena
Belčič Mikič, Tanja
Debeljak, Nataša
Preložnik Zupan, Irena
author_facet Anžej Doma, Saša
Drnovšek, Eva
Kristan, Aleša
Fink, Martina
Sever, Matjaž
Podgornik, Helena
Belčič Mikič, Tanja
Debeljak, Nataša
Preložnik Zupan, Irena
author_sort Anžej Doma, Saša
collection PubMed
description Erythrocytosis has a diverse background. While polycythaemia vera has well defined criteria, the diagnostic approach and management of other types of erythrocytosis are more challenging. The aim of study was to retrospectively analyse the aetiology and management of non-clonal erythrocytosis patients referred to a haematology outpatient clinic in an 8-year period using a 3-step algorithm. The first step was inclusion of patients with Hb > 185 g/L and/or Hct > 0.52 in men and Hb > 165 g/L and/or Hct > 0.48 in women on two visits ≥ two months apart, thus confirming true erythrocytosis. Secondly, polycythaemia vera was excluded and secondary causes of erythrocytosis (SE) identified. Thirdly, idiopathic erythrocytosis patients (IE) were referred to next-generation sequencing for possible genetic background evaluation. Of the 116 patients, 75 (65%) are men and 41 (35%) women, with non-clonal erythrocytosis 34/116 (29%) had SE, 15/116 (13%) IE and 67/116 (58%) stayed incompletely characterized (ICE). Patients with SE were significantly older and had significantly higher Hb and Hct compared to patients with IE. Most frequently, SE was attributed to obstructive sleep apnoea and smoking. Phlebotomies were performed in 56, 53 and 40% of patients in the SE, IE, and ICE group, respectively. Approx. 70% of patients in each group received aspirin. Thrombotic events were registered in 12, 20 and 15% of SE, IE and ICE patients, respectively. Congenital erythrocytosis type 4 (ECYT4) was diagnosed in one patient. The study demonstrates real-life management of non-clonal erythrocytosis which could be optimized using a 3-step diagnostic algorithm.
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spelling pubmed-82853332021-07-20 Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience Anžej Doma, Saša Drnovšek, Eva Kristan, Aleša Fink, Martina Sever, Matjaž Podgornik, Helena Belčič Mikič, Tanja Debeljak, Nataša Preložnik Zupan, Irena Ann Hematol Original Article Erythrocytosis has a diverse background. While polycythaemia vera has well defined criteria, the diagnostic approach and management of other types of erythrocytosis are more challenging. The aim of study was to retrospectively analyse the aetiology and management of non-clonal erythrocytosis patients referred to a haematology outpatient clinic in an 8-year period using a 3-step algorithm. The first step was inclusion of patients with Hb > 185 g/L and/or Hct > 0.52 in men and Hb > 165 g/L and/or Hct > 0.48 in women on two visits ≥ two months apart, thus confirming true erythrocytosis. Secondly, polycythaemia vera was excluded and secondary causes of erythrocytosis (SE) identified. Thirdly, idiopathic erythrocytosis patients (IE) were referred to next-generation sequencing for possible genetic background evaluation. Of the 116 patients, 75 (65%) are men and 41 (35%) women, with non-clonal erythrocytosis 34/116 (29%) had SE, 15/116 (13%) IE and 67/116 (58%) stayed incompletely characterized (ICE). Patients with SE were significantly older and had significantly higher Hb and Hct compared to patients with IE. Most frequently, SE was attributed to obstructive sleep apnoea and smoking. Phlebotomies were performed in 56, 53 and 40% of patients in the SE, IE, and ICE group, respectively. Approx. 70% of patients in each group received aspirin. Thrombotic events were registered in 12, 20 and 15% of SE, IE and ICE patients, respectively. Congenital erythrocytosis type 4 (ECYT4) was diagnosed in one patient. The study demonstrates real-life management of non-clonal erythrocytosis which could be optimized using a 3-step diagnostic algorithm. Springer Berlin Heidelberg 2021-05-19 2021 /pmc/articles/PMC8285333/ /pubmed/34013406 http://dx.doi.org/10.1007/s00277-021-04546-4 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Anžej Doma, Saša
Drnovšek, Eva
Kristan, Aleša
Fink, Martina
Sever, Matjaž
Podgornik, Helena
Belčič Mikič, Tanja
Debeljak, Nataša
Preložnik Zupan, Irena
Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title_full Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title_fullStr Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title_full_unstemmed Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title_short Diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
title_sort diagnosis and management of non-clonal erythrocytosis remains challenging: a single centre clinical experience
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8285333/
https://www.ncbi.nlm.nih.gov/pubmed/34013406
http://dx.doi.org/10.1007/s00277-021-04546-4
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