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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Polycythemias or erythrocytoses in childhood and adolescence are very rare. Systematic data on the clinical presentation and laboratory evaluations as well as on treatment regimens are sparse. The diagnostic program in absolute erythrocytosis includes extensive clinical, hematological, biochemical, and molecular biological examinations which should be applied following a stepwise algorithm. Absolute erythrocytoses are usually subdivided into primary and secondary forms. Primary erythrocytosis is a condition in which the erythropoietic compartment is expanding independently of extrinsic influences or by responding inadequately to them. Primary erythrocytoses include primary familial and congenital polycythemia (PFCP) due to mutations of the erythropoietin (Epo) receptor gene and the myeloproliferative disorder polycythemia vera. Secondary erythrocytoses are driven by hormonal factors (predominantly by Epo) extrinsic to the erythroid compartment. The increased Epo secretion may represent either a physiologic response to tissue hypoxia, an abnormal autonomous Epo production, or a dysregulation of the oxygen-dependent Epo synthesis. Congenital secondary erythrocytoses are caused, e.g., by hemoglobin variants with increased oxygen affinity, by 2,3-bisphosphoglycerate deficiency, or by mutations in the von Hippel-Lindau gene associated with a disturbed oxygen-dependent regulation of Epo synthesis.
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PMID:Childhood polycythemias/erythrocytoses: classification, diagnosis, clinical presentation, and treatment. 1559 50

Primary erythrocytosis, or polycythemia vera, is a myeloproliferative disease caused by the exaggerated increase of erythroid precursor cells in the bone marrow. We report the case of an 11-year-old male mixed-breed dog that had tachypnea and spastic tetraplegia. There was a significant increase in hematocrit. After phlebotomy and fluid therapy, the dog's condition improved. A diagnosis of primary erythrocytosis was supported by serum levels of erythropoietin. The dog responded well to treatment with administration of hydroxyurea (15 mg/kg), phlebotomies, and fluid therapy. However, after 18 months, he had an acute recurrence of clinical signs and was euthanized. We observed that long-term maintenance with hydroxyurea at a dosage of 15 mg/kg every 48 hours was adequate for managing polycythemia vera, with a survival time of 18 months in the present case. However, longer dose intervals are likely not appropriate. We believe that this may be helpful to other veterinarians facing the same problems in the treatment of polycythemia vera.
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PMID:Diagnosis and Treatment of Primary Erythrocytosis in a Dog: A Case Report. 2635 27