Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2007 Dec;22(12):1775-7.
doi: 10.1007/s11606-007-0356-4. Epub 2007 Oct 5.

Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis

Shelonitda S Rose  1 , Ashish A Shah, Donald R Hoover, Parvin Saidi
Affiliations
Case Reports

Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis

Shelonitda S Rose et al. J Gen Intern Med. 2007 Dec.

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.

PubMed Disclaimer

References

    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '15477203', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/15477203/'}]}
    2. Mossuz P, Girodon F, Donnard M, Latger-Cannard V, Dobo I, Boiret N, et al. Diagnostic value of serum erythropoietin level in patients with absolute erythrocytosis. Haematologica. 2004;89(10):1194–8. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '4827655', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/4827655/'}]}
    2. Prchal JF, Axelrad AA. Bone-marrow responses in polycythemia vera. N Engl J Med. 1974;290:1382. - PubMed
    1. None
    2. Warrell DA, Cox TM, Firth JD, Benz EJ. Cyanotic Heart Disease: A Multisystem Disorder. Oxford Textbook of Medicine, 4th edition. London: Oxford University Press; 2003.
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PubMed', 'value': '1856391', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/1856391/'}]}
    2. Territo MC, Rosove MH. Cyanotic congenital heart disease: hematologic management. J Am Coll Cardiol. 1991;18(2):320–2. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'PMC', 'value': 'PMC289383', 'is_inner': False, 'url': 'https://pmc.ncbi.nlm.nih.gov/articles/PMC289383/'}, {'type': 'PubMed', 'value': '13936779', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/13936779/'}]}
    2. Murray JF, Gold P, Johnson BL Jr. The circulatory effects of hematocrit variations in normovolemic and hypervolemic dogs. J Clin Invest. 1963;42:1150–9. - PMC - PubMed

Publication types