Significance of, and Difficulty in Diagnosing, Erythropoietin Deficiency in Sickle Cell Anemia
| Autor: | Santosh L. Saraf, Michael Gowhari, Lani Krauz, Joseph DeSimone, Stephen Vara, Seema Sidhwani, Louise Dorn, Robert E. Molokie, Yogen Saunthararajah, Giovanni Infusino, Mohammed Farooqui |
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| Rok vydání: | 2008 |
| Předmět: |
Hemolytic anemia
medicine.medical_specialty business.industry Reticulocytosis Anemia Immunology Renal function Cell Biology Hematology medicine.disease Biochemistry Gastroenterology Sickle cell anemia Erythropoietin Internal medicine Medicine Reticulocytopenia medicine.symptom business Vaso-occlusive crisis medicine.drug |
| Zdroj: | Blood. 112:2479-2479 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v112.11.2479.2479 |
| Popis: | The clinical diagnosis of erythropoietin deficiency is usually prompted by new onset anemia on a background of elevated serum creatinine, and is then confirmed by measurement of serum erythropoietin. In patients with sickle cell anemia (SCA), who are already anemic, and in whom base-line creatinine and erythropoietin levels tend to run low, it is easy to miss this diagnosis. We demonstrate the clinical significance of erythropoietin deficiency in SCA and suggest alternative criteria for considering this diagnosis. Compensatory reticulocytosis maintains hemoglobin levels compatible with life in the chronic hemolytic anemia of SCA. Relative reticulocytopenia (RR) has been defined by the Multi-Center Study of Hydroxyurea Follow-Up Study (MSH-FU) as reticulocytes SCA causes multi-organ damage, therefore, RR could result from decreased bone marrow function, from renal damage causing a decrease in erythropoietin levels, or from both processes. In univariate analyses, RR was significantly associated with thrombocytopenia, leucopenia, elevated serum creatinine and proteinuria (p Importantly, the erythropoietin/reticulocyte ratios that implicated erythropoietin deficiency in the etiology of RR were mostly noted in patients who did not meet standard clinical definitions of chronic renal failure: < 30% of the patients with RR had serum creatinine levels >1.1 or calculated creatinine clearance In conclusion, RR in adult SCA is not associated with more frequent vaso-occlusive crises but nonetheless has a major association with mortality. An important and potentially correctable cause of RR is erythropoietin deficiency, which can be diagnosed in patients with early renal damage manifest by proteinuria but without azotemia. This sub-set of SCA patients requires more study, including evaluation for the causes of death, and the possible benefits of erythropoietin replacement therapy, using end-point criteria such as survival or progression of chronic organ damage (for eg., left-ventricular dysfunction) rather than vaso-occlusive crisis frequency. |
| Databáze: | OpenAIRE |
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