Abstract: The Alpha haemoglobin-stabilising protein (AHSP) is a key chaperone molecule synthesised in red blood cell (RBC) precursors. In this study, the inventors have developed a sandwich ELISA method which allows the quantification of AHSP protein in RBCs of normal subjects and in sickle cell anaemia (SCA) patients treated or untreated by hydroxycarbamide. AHSP was significantly more abundant in untreated SCA patients than in controls (p<0.0001). After hydroxycarbamide treatment, the AHSP decreased but remained significantly more abundant than in controls. A highly positive correlation was found between AHSP concentration and the ?-Hb pool. So, like the ?-Hb pool, the AHSP concentration could be a new and better RBC biomarker of SCA disease, much more easily measurable than the ?-Hb pool.

Abstract: The present invention relates to a method for determining the quality of haemoglobin (Hb) during the storage period of red blood cell (RBC) units comprising a step of detecting soluble alpha-haemoglobin (?-Hb) pool in RBC lysates and concluding that the presence of ?-Hb pool indicates a conservation of quality of Hb during the storage RBCs. Inventors have determined the impact of RBC units aging on the quality of Hb and on the soluble ?-Hb pool level in RBCs. For this purpose, 21 RBC units were collected, stored at +4 to 6° C. and samples were taken at two different storage times (D3-D8 and D38-D42) to evaluate spectral characteristics of Hb and soluble ?-Hb pool in RBCs. Two additional samples were collected from 16 RBC units, at intermediate time points during storage (D13-D17 and D24-D29; n=16). The ?-Hb dosing assay uses the specific character of the interaction between the ?-Hb and the AHSP, the ? chaperone, to trap the ?-Hb present in the RBC lysates of RBC units.

Abstract: The invention relates to a method for diagnosing, staging and/or monitoring a hemoglobin-related disorder such as ?-thalassemia or a treatment against said hemoglobin-related disorder in a subject in need thereof based on the detection and/or quantification the presence of free ?-Hb pool in a biological sample obtained from said subject.

Abstract: The invention relates to a method for diagnosing, staging and/or monitoring a hemoglobin-related disorder such as ?-thalassemia or a treatment against said hemoglobin-related disorder in a subject in need thereof based on the detection and/or quantification the presence of free ?-Hb pool in a biological sample obtained from said subject.

Abstract: A method for diagnosing and/or staging a hemoglobin disorder such as ?-thalassemia involves contacting a blood sample of a subject with an Alpha-Hemoglobin Stabilizing Protein which may be present on a solid support, and detecting and/or quantifying free ?-Hb. Detection or quantification can be achieved by photometry such as HTRF or immunological procedures such as EIA and ELISA. The free ?-Hb is compared to a reference value, and can be used to correlate with the diagnosis and/or staging of the hemoglobin disorder for the subject.

Abstract: The invention relates to a method for diagnosing, staging and/or monitoring a hemoglobin-related disorder such as ?-thalassemia or a treatment against said hemoglobin-related disorder in a subject in need thereof based on the detection and/or quantification the presence of free ?-Hb pool in a biological sample obtained from said subject.