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Lancet (London, England) Jul 1989
Review
Topics: Blood Viscosity; Bloodletting; Evaluation Studies as Topic; Heart Failure; Hematocrit; Humans; Hypoxia; Lung Diseases, Obstructive; Polycythemia
PubMed: 2567796
DOI: 10.1016/s0140-6736(89)90259-6 -
British Journal of Haematology Mar 2010Primary familial and congenital polycythemia (PFCP) is an autosomal-dominant proliferative disorder characterized by erythrocytosis and hypersensitivity of erythroid... (Review)
Review
Primary familial and congenital polycythemia (PFCP) is an autosomal-dominant proliferative disorder characterized by erythrocytosis and hypersensitivity of erythroid progenitors to erythropoietin (Epo). Several lines of evidence suggest a causal role of truncated erythropoietin receptor (EpoR) in this disease. In this review, we discuss PFCP in the context of erythrocytosis and EpoR signalling. We focus on recent studies describing mechanisms underlying Epo-dependent EpoR down-regulation. One mechanism depends on internalization mediated through the p85 regulatory subunit of the Phosphoinositide 3-Kinase, and the other utilizes ubiquitin-based proteasomal degradation. Truncated PFCP EpoRs are not properly down-regulated upon stimulation, underscoring the importance of these mechanisms in the pathogenesis of PFCP.
Topics: Down-Regulation; Humans; Mutation; Polycythemia; Receptors, Erythropoietin; Signal Transduction
PubMed: 20096014
DOI: 10.1111/j.1365-2141.2009.08069.x -
The Practitioner Apr 1993
Topics: Aged; Bloodletting; Hematocrit; Humans; Middle Aged; Polycythemia
PubMed: 8351279
DOI: No ID Found -
The Practitioner Jan 1982
Topics: Adult; Diagnosis, Differential; Erythropoietin; Humans; Polycythemia; Polycythemia Vera; Prognosis
PubMed: 7079225
DOI: No ID Found -
Lancet (London, England) Apr 1959
Topics: Humans; Polycythemia
PubMed: 13642910
DOI: No ID Found -
Nursing Times Dec 1976
Topics: Humans; Polycythemia
PubMed: 1005135
DOI: No ID Found -
The Practitioner Jun 1970
Topics: Blood Cell Count; Female; Hematocrit; Humans; Male; Phosphorus Isotopes; Polycythemia; Prognosis
PubMed: 5452204
DOI: No ID Found -
Nursing Times Mar 1964
Topics: Aged; Humans; Polycythemia; Polycythemia Vera
PubMed: 14130801
DOI: No ID Found -
Schweizerische Medizinische... Feb 1969
Topics: Anticoagulants; Child; Diagnosis, Differential; Female; Humans; Myeloproliferative Disorders; Neurologic Manifestations; Polycythemia; Polycythemia Vera; Pyrimethamine
PubMed: 5781368
DOI: No ID Found -
Presse Medicale (Paris, France : 1983) Dec 1992Between 1971 and 1990, 251 kidney transplanted patients with a well functioning graft were evaluated to determine the frequency of post-transplant erythrocytosis (PTE)....
Between 1971 and 1990, 251 kidney transplanted patients with a well functioning graft were evaluated to determine the frequency of post-transplant erythrocytosis (PTE). Thirty-one patients (13 percent) developed polycythaemia 10.6 +/- 10 months after transplantation. Thromboembolic complications occurred in 22 percent of the cases. The frequency of PTE was higher in males than in females (sex ratio: 7.2 vs 2.1; P < 0.05). Patients with renal dysplasia had a lower incidence of PTE (3 vs 22 percent; P < 0.05) as did those who had been treated with azathioprine (9.4 vs 19 percent; P < 0.05). None of the patients treated with recombinant erythropoietin before transplantation developed PTE during a mean follow-up of 15.1 +/- 4.5 months. The majority of polycythaemic patients had normal erythropoietin levels. These results show that there is an erythropoietin-independent proliferation due to an increased sensitivity of erythroid progenitors or to an erythroid stem cell stimulation by cytokines.
Topics: Adult; Azathioprine; Erythropoietin; Female; Humans; Kidney Transplantation; Male; Middle Aged; Polycythemia; Postoperative Complications; Retrospective Studies; Thromboembolism
PubMed: 1294956
DOI: No ID Found