Title: A study on C-reactive protein as an early marker of vasococclusive crisis in homozygous sickle cell disease (HbSS) and sickle cell-b thalassemia disease (Hb S-b thal)

Authors: Dr Kausik Goswami, Dr Sheela Kandulna (Goswami), Dr Anindya Sasmal, Dr Prakash Chandra Panda, Dr I R Agrawalla, Dr Sukanta Sen

 DOI: https://dx.doi.org/10.18535/jmscr/v6i10.230

Abstract

Background:  Sickle cell disease (SCD) is associated with a chronic inflammatory state. SCD patients often seek care in the Emergency Department (ED) due to vaso-occlusive crisis (VOC), the most common complication of SCD. Currently, no diagnostic test can determine if a SCD patient is having an acute VOC. Methodology: Irrespective of the clinical diagnosis and type of sickle cell crises the confirmation of haemoglobin pattern in sickle cell disease patients and healthy subjects were done by sickling test, hemoglobin electrophoresis or high performance liquid chromatography.

Results: CRP status in Group I (Hb electrophoretically AA) healthy subjects were analyzed. It showed that irrespective of their age and sex 100% were CRP negative during their healthy state. Diarrhoea (26%), LRTI (16%), URTI (14%) and malaria (12%) were more prevalent infection in sickle cell patients in the present study. It was observed that out of 21 patients with CRP positivity in early phase 17 patients (80.9%) developed vaso-occlusive crisis during their follow up.

Conclusion: The present study demonstrated a strong association between the inflammatory biomarker hs-CRP and vasococclusive crisis in homozygous sickle cell disease (HbSS) and sickle cell-b thalassemia disease (Hb S-b thal). We believe these results highlight the clinical relevance of inflammation in microvessel occlusive complications, and provide a basis for the study of hs-CRP as a potential biomarker for predictive modelling of clinical outcomes in SCD.

Keywords: Sickle Cell Disorder, homozygous sickle cell disease (HbSS), sickle cell-b thalassemia disease, vaso-occlusive crisis, hemoglobin electrophoresis, biomarker, C-reactive protein.

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Corresponding Author

Dr Sheela Kandulna (Goswami)

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