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Erythrocytosis in a scleroderma patient
Eric Mabo1, Timi Edeki, Gloria E Westney
1Department of Internal Medicine, Morehouse School of Medicine, Atlanta, GA 30310, USA.
The Mount Sinai Journal of Medicine, New York
|June 8, 2006
Summary
Patients with scleroderma lung disease and high red blood cell counts (erythrocytosis) may appear to have normal oxygen levels during the day. Monitoring oxygen during sleep and checking erythropoietin levels are crucial for diagnosis.
Area of Science:
- Pulmonary Medicine
- Hematology
Background:
- Scleroderma lung disease can lead to complications like erythrocytosis.
- Erythrocytosis, or an abnormally high red blood cell count, can cause hyperviscosity symptoms.
Observation:
- A patient with scleroderma lung disease and severe erythrocytosis presented with headache and blurry vision.
- Despite nocturnal hypoxemia, daytime resting oxygen saturation was normal.
- Initial serum erythropoietin levels were unexpectedly low.
Findings:
- Phlebotomy improved hyperviscosity symptoms, reducing hemoglobin from 22.3 to 18.3 g/dL.
- Repeat erythropoietin levels normalized or increased.
- This case highlights that patients with chronic interstitial lung disease and erythrocytosis may exhibit normoxemia at rest and variable erythropoietin levels.
Implications:
- Diagnosing erythrocytosis in chronic interstitial lung disease requires monitoring oxygen saturation during sleep and exertion.
- Consider repeat erythropoietin sampling.
- Continuous oxygen therapy is recommended for these patients, even without apparent daytime hypoxemia.
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