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Evaluation of the relationship between intravascular hemolysis and clinical manifestations in sickle cell disease: decreased hemopexin during vaso-occlusive crises and increased inflammation in acute chest syndrome

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Abstract

The aims of this study were to determine the possible relationships between the levels of hemin, hemopexin, acid sphingomyelinase, nitrite/nitrate (NOx), and other parameters in patients with SCD and to assess whether they were associated with vaso-occlusive crises (VOCs) or acute chest syndrome (ACS). Patients with SCD (homozygous or sickle beta-thalassemia) who were confirmed to have VOC or ACS were included. Blood samples were obtained at admission, on the third day of hospitalization, and at steady state. Demographic characteristics, pain (visual analog scale), complication history, complete blood count, lactate dehydrogenase, and C-reactive protein levels were recorded. Hemin, hemopexin, acid sphingomyelinase, and NOx were measured via ELISA. A total of 31 patients (22 VOC, 9 ACS) were included. Mean age was 16.4 ± 4.7 years. Admission white blood cell count and C-reactive protein levels were significantly higher in the ACS group. Patients with ACS also demonstrated a significant decreasing trend of LDH and an increasing trend of NOx values from admission to steady state. Notably, hemopexin levels were significantly lower on the third day of hospitalization compared to steady-state levels. Despite limited patient count in the ACS group, these patients appear to have strikingly greater inflammatory activation at admission, and the progression of ACS may be associated with LDH and NOx levels. Lower hemopexin levels during hospitalization versus steady state appear to support a role for the administration of hemopexin therapy during crises.

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Authors and Affiliations

  1. Department of Pediatric Hematology, Faculty of Medicine, Mersin University, Mersin, Turkey

    Nazim Yildirim, Selma Unal & Feryal Karahan

  2. Department of Medical Biochemistry, Faculty of Medicine, Hacettepe University, 06100 Sıhhiye, Ankara, Turkey

    Ahmet Yalcinkaya & Yesim Oztas

Authors
  1. Nazim Yildirim
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  2. Selma Unal
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  3. Ahmet Yalcinkaya
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  4. Feryal Karahan
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  5. Yesim Oztas
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Corresponding author

Correspondence to Ahmet Yalcinkaya.

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Ethics approval

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008 (5). Ethical approval for the study was obtained from the Clinical Research Ethics Committee of Mersin University Faculty of Medicine (Decision number: 78017789/050.01.04/529310).

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Informed consent was obtained from all patients for being included in the study. All patients or their caretakers signed written informed consent forms when they were admitted to the ward.

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The authors declare no competing interests.

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Yildirim, N., Unal, S., Yalcinkaya, A. et al. Evaluation of the relationship between intravascular hemolysis and clinical manifestations in sickle cell disease: decreased hemopexin during vaso-occlusive crises and increased inflammation in acute chest syndrome. Ann Hematol 101, 35–41 (2022). https://doi.org/10.1007/s00277-021-04667-w

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  • DOI: https://doi.org/10.1007/s00277-021-04667-w

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