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Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis.
J Gen Intern Med 2007; 22(12):1775-7JG

Abstract

Secondary erythrocytosis of cyanotic congenital heart disease (CCHD) is pathologically different from primary erythrocytosis of polycythemia vera (PV). An association between elevated hematocrit and thrombosis has been established in PV patients, and treatment guidelines recommend maintaining hematocrit <45%. Although an association between elevated hematocrit and thrombosis has not been established in CCHD and secondary erythrocytosis, the current clinical practice is to phlebotomize these patients to hematocrit <65%. We report a 21-year-old woman with CCHD who presented with symptomatic erythrocytosis with numbness and tingling with hemoglobin 25.2 g/dl and hematocrit 75.8%. Her symptoms resolved with IV hydration. Other factors, including dehydration and iron deficiency, may precipitate hyperviscosity symptoms. The treatment is volume replacement and low-dose iron therapy, not phlebotomy. Repeated phlebotomy causes iron deficiency with microcytic erythrocytes, which increases the whole blood viscosity and, therefore, can potentially accentuate rather than decrease the risk for a cerebrovascular accident.

Authors+Show Affiliations

Division of Hematology, Department of Medicine, UMDNJ/Robert Wood Johnson Medical School, New Brunswick, NJ, USA. rosesh@umdnj.eduNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

17917783

Citation

Rose, Shelonitda S., et al. "Cyanotic Congenital Heart Disease (CCHD) With Symptomatic Erythrocytosis." Journal of General Internal Medicine, vol. 22, no. 12, 2007, pp. 1775-7.
Rose SS, Shah AA, Hoover DR, et al. Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis. J Gen Intern Med. 2007;22(12):1775-7.
Rose, S. S., Shah, A. A., Hoover, D. R., & Saidi, P. (2007). Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis. Journal of General Internal Medicine, 22(12), pp. 1775-7.
Rose SS, et al. Cyanotic Congenital Heart Disease (CCHD) With Symptomatic Erythrocytosis. J Gen Intern Med. 2007;22(12):1775-7. PubMed PMID: 17917783.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis. AU - Rose,Shelonitda S, AU - Shah,Ashish A, AU - Hoover,Donald R, AU - Saidi,Parvin, Y1 - 2007/10/05/ PY - 2007/02/26/received PY - 2007/08/16/accepted PY - 2007/06/21/revised PY - 2007/10/6/pubmed PY - 2008/1/11/medline PY - 2007/10/6/entrez SP - 1775 EP - 7 JF - Journal of general internal medicine JO - J Gen Intern Med VL - 22 IS - 12 N2 - Secondary erythrocytosis of cyanotic congenital heart disease (CCHD) is pathologically different from primary erythrocytosis of polycythemia vera (PV). An association between elevated hematocrit and thrombosis has been established in PV patients, and treatment guidelines recommend maintaining hematocrit <45%. Although an association between elevated hematocrit and thrombosis has not been established in CCHD and secondary erythrocytosis, the current clinical practice is to phlebotomize these patients to hematocrit <65%. We report a 21-year-old woman with CCHD who presented with symptomatic erythrocytosis with numbness and tingling with hemoglobin 25.2 g/dl and hematocrit 75.8%. Her symptoms resolved with IV hydration. Other factors, including dehydration and iron deficiency, may precipitate hyperviscosity symptoms. The treatment is volume replacement and low-dose iron therapy, not phlebotomy. Repeated phlebotomy causes iron deficiency with microcytic erythrocytes, which increases the whole blood viscosity and, therefore, can potentially accentuate rather than decrease the risk for a cerebrovascular accident. SN - 1525-1497 UR - https://www.unboundmedicine.com/medline/citation/17917783/Cyanotic_congenital_heart_disease_(CCHD)_with_symptomatic_erythrocytosis L2 - https://dx.doi.org/10.1007/s11606-007-0356-4 DB - PRIME DP - Unbound Medicine ER -