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Levels of tumor necrosis factor alpha/cachectin (TNF alpha) in sera from patients with sickle cell disease
I Malavé1, Y Perdomo, E Escalona
1Center of Experimental Medicine, Instituto Venezolano de Investigaciones Científicas, Caracas, Venezuela.
Insights
Adult sickle cell disease (SCD) patients show elevated tumor necrosis factor alpha (TNF alpha) levels. Higher TNF alpha in SCD correlates with lower fetal hemoglobin (Hb F) percentages, suggesting a link between inflammation and disease severity.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- Tumor necrosis factor alpha (TNF alpha), a pro-inflammatory cytokine, plays a role in various inflammatory conditions.
Purpose of the Study:
- To investigate serum levels of TNF alpha in adult patients with sickle cell disease (SCD).
- To explore the relationship between TNF alpha levels and hemoglobin variants (SS and SC hemoglobinopathies) in SCD patients.
- To examine the correlation between TNF alpha levels and fetal hemoglobin (Hb F) percentages in SCD patients.
Main Methods:
- Serum samples were collected from 31 SS and 10 SC hemoglobinopathy patients, along with matched controls.
- Patients were in a non-crisis state for at least 4 weeks prior to sample collection.
- TNF alpha levels were quantified using an immunoenzymatic assay with a detection limit of 25 pg/ml.
Main Results:
- Significantly higher percentages of detectable TNF alpha were found in SCD patients compared to controls.
- Mean TNF alpha levels were significantly elevated in the overall SCD group, as well as in SS and SC hemoglobinopathy subgroups, versus controls.
- An inverse correlation was observed between serum TNF alpha levels and the percentage of Hb F in SCD patients.
Conclusions:
- Adult SCD patients exhibit increased serum levels of TNF alpha.
- Elevated TNF alpha in SCD may be linked to disease pathophysiology and potentially modulated by Hb F levels.
Abstract:
Serum levels of tumor necrosis factor alpha/cachectin (TNF alpha) were studied in a group of adult patients with sickle cell disease (SCD), which include 31 patients with homozygous SS hemoglobinopathy and 10 patients bearing double heterozygous SC hemoglobinopathy and in their matched normal controls. All patients tested did not show any form of crisis for at least 4 weeks prior to the extraction of the sample. The amount of TNF alpha in serum was quantitated by means of an immunoenzymatic assay with a lower limit of detection of 25 pg/ml. The percentage of sera with detectable levels of TNF alpha was significantly increased in SCD patients as compared with the normal controls. Mean TNF alpha values in individuals with detectable levels of the cytokine were also significantly higher in the whole group of SCD patients and in patients bearing either SS or SC hemoglobinopathies than in the control group. An inverse correlation was observed between the percentages of Hb F and the levels of TNF alpha found in the sera from the patients.

