AI Article Synopsis

  • Erythrocytosis has various causes, and distinguishing between them is crucial, especially since polycythemia vera has clear criteria while other types do not.
  • A study analyzed patients with non-clonal erythrocytosis over eight years using a three-step algorithm that confirmed true cases and identified secondary causes.
  • Of the 116 patients studied, the majority were male, with 29% having secondary erythrocytosis, 13% idiopathic erythrocytosis, and 58% remaining incompletely characterized; common causes included sleep apnea and smoking, with significant treatment variations observed among patient groups.

Article Abstract

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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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8285333PMC
http://dx.doi.org/10.1007/s00277-021-04546-4DOI Listing

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