Laboratory profile of sickle cell disease: a cross-sectional analysis. The Cooperative Study of Sickle Cell Disease
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This study analyzed laboratory data from over 2600 patients with sickle cell disease (SCD). Findings reveal age and sex-related variations in packed cell volume, leukocyte, and platelet counts, alongside progressive renal function decline in SCD patients.
Area of Science:
- Hematology
- Pediatrics
- Nephrology
Background:
- Sickle cell disease (SCD) encompasses several inherited blood disorders, including sickle cell anemia (HbSS), HbSC disease, and HbS-beta(+)-thalassemia.
- Understanding baseline laboratory values in SCD patients is crucial for managing the condition and evaluating treatment efficacy.
Purpose of the Study:
- To establish reference ranges for key laboratory parameters in a large cohort of SCD patients.
- To describe age- and sex-specific trends in packed cell volume, leukocyte counts, platelet counts, and creatinine levels.
Main Methods:
- Analysis of steady-state laboratory data from over 2600 patients aged 2 years and older with HbSS, HbSC, and HbS-beta(+)-thalassemia.
- Evaluation of packed cell volume (PCV), leukocyte count, platelet count, and creatinine levels in relation to age and sex.
Main Results:
- Packed cell volume (PCV) shows distinct age- and sex-dependent patterns, with lower levels in younger males and a decline after age 40 in HbSS.
- Leukocyte counts are elevated in HbSS, potentially limiting infection assessment, while HbSC and HbS-beta(+)-thalassemia patients often have normal counts.
- Platelet counts in HbSS tend to be elevated and decrease with age; creatinine levels show a progressive rise, particularly after age 14 in HbSS, indicating renal function deterioration.
Conclusions:
- Established laboratory data provide a benchmark for assessing disease progression and treatment effects in sickle cell disease.
- Age and sex significantly influence hematological parameters in SCD, necessitating age- and sex-specific interpretations.
- Progressive renal function decline is evident in SCD patients over 20, likely due to the disease's impact on kidney health.
Abstract:
We have collected steady-state laboratory data for over 2600 patients, age 2 years and over, with sickle cell anemia (HbSS), HbSC disease, and HbS-beta(+)-thalassemia. The packed cell volume (PCV) is lower in males than in females until 17 or 18 years of age in HbSS and ages 13 to 15 in HbSC, but then becomes consistently higher in males. After age 40, the PCV falls in HbSS. The steady-state leukocyte count in HbSS is higher than that in normals, blunting the utility of this measurement in the assessment of infection. In HbSC and HbS-beta(+)-thalassemia, the leukocyte counts are more often within the range of normal. Platelet counts in HbSS are often found to be above normal and show a downward trend with age. There is a progressive rise in creatinine with age. In HbSS, this rise begins at age 14 and may be accounted for by the increased muscle mass that occurs with puberty. The further deterioration of renal function in patients over 20 may be a result of the known adverse effects of sickle cell disease upon the kidney. Our data provide a basis to compare perturbations caused by intercurrent complications and new therapies, as well as to contrast with similar information from other populations of patients with sickle cell disease.