Document Detail

Idiopathic erythrocytosis, diagnosis and clinical management.
MedLine Citation:
PMID:  11317965     Owner:  NLM     Status:  MEDLINE    
By definition, idiopathic erythrocytosis (IE) applies to a group of patients characterised by having a measured RCM above their predicted normal range (an absolute erythrocytosis) and following investigation do not have a form of primary or secondary erythrocytosis. Patients with IE are heterogenous. The possibilities include physiological variation, 'early' polycythaemia vera (10-15% develop clear features of PV over a few years), unrecognized congenital erythrocytosis, unrecognized or unrecognizable secondary acquired erythrocytosis or a currently undescribed form of primary or secondary erythrocytosis. Patients are more commonly male with a median age at presentation of 55-60 years. Approximately half of the patients present with vascular occlusive complications. Retrospective evidence indicates that vascular occlusion occurs less frequently when the PCV is controlled at normal levels. Venesection is the treatment of choice to lower the PCV. As a general approach to management, all patients with a PCV above 0.54 should be venesected to a PCV less than 0.45. This target PCV should also apply to patients with lesser degrees of raised PCV who have additional other risk factors for vascular occlusion.
T C Pearson; M Messinezy
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Pathologie-biologie     Volume:  49     ISSN:  0369-8114     ISO Abbreviation:  Pathol. Biol.     Publication Date:  2001 Mar 
Date Detail:
Created Date:  2001-04-24     Completed Date:  2001-06-14     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0265365     Medline TA:  Pathol Biol (Paris)     Country:  France    
Other Details:
Languages:  eng     Pagination:  170-7     Citation Subset:  IM    
Department of Haematological Medicine, Guy's, King's and St Thomas' School of Medicine, St Thomas' Hospital, London SE1 7EH, UK.
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MeSH Terms
Anoxia / complications
Arterial Occlusive Diseases / etiology
Bone Marrow / pathology
Chlorambucil / adverse effects,  therapeutic use
Diagnosis, Differential
Endocrine System Diseases / complications
Erythrocyte Volume
Erythroid Precursor Cells / pathology
Erythropoietin / blood
Genetic Predisposition to Disease
Kidney Diseases / complications
Leukemia / chemically induced
Leukemia, Radiation-Induced / etiology
Middle Aged
Phosphorus Radioisotopes / adverse effects,  therapeutic use
Polycythemia* / classification,  congenital,  diagnosis,  etiology,  therapy
Polycythemia Vera / diagnosis
Receptors, Erythropoietin / deficiency,  genetics
Sequence Deletion
Smoking / blood
Stroke / etiology
Reg. No./Substance:
0/Phosphorus Radioisotopes; 0/Receptors, Erythropoietin; 11096-26-7/Erythropoietin; 305-03-3/Chlorambucil

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