Sickle cell disease

Sickle cell disease is the world’s number one genetic disorder, affecting up to 8 million people and 515 000 newborns every year. Sickle cell anemia alters the structure of hemoglobin, the main oxygen-carrying protein in red blood cells. This anomaly is responsible for the rigidification of red blood cells, generating chronic anemia, increased risk of infection and vaso-occlusive crises (CVO). The latter is the major symptom of sickle cell disease, and its most physically, emotionally and socio-economically disabling manifestation. CVO are extremely painful and unpredictable, and they can be lethal, as in the case of acute lung complications, which are the leading cause of death in sickle cell patients. Long-term complications of the disease, notably organ failure caused by repeated crises, greatly reduce life expectancy (40 to 60 years in developed countries) and quality of life for patients.

ICOVELL’s has the ambition to improve patients care by providing :

A marker of clinical follow-up

An indicator of patient compliance and treatment efficacy

A predictor of disease severity

A predicator of imminent vaso-occlusive crises

fTT correlates with crises parameters, hemolysis rate pain intensity and hypertensive drugs

The fTT marker, a good candidate for predicting vaso-occlusive crises

(A) Comparison of f TT values of samples from patients with sickle cell disease (HbSS) and samples from healthy donors (HbAA) and healthy mutation carriers (HbAS). n = number of samples analyzed. (B) Mean TT marker value for 19 sickle cell patients, the number of samples is indicated above each column (mean +/- standard deviation). (C) Evolution of the TT marker over time outside the crisis period and during a vaso-occlusive crisis. The dotted line represents the mean value; the shaded area is 2 standard deviations wide around the mean value. The values of the TT marker during the crisis and outside the crisis period are shown in red and black respectively.