Table 5 Characteristics of the patients followed in Mayotte according to the HbF level
Profile | HbF ≥ 10% | HbF < 10% | OR (95%CI) | P | P* |
|---|---|---|---|---|---|
Age, Mean (SD) | 8,6 (±0,5) | 12,1 (±0,6) | < 0,001 | 0.09 | |
Hemoglobin sickle cell genotype n (%) | 0.187 | 0.2 | |||
HbSS | 58 (68,2) | 60 (76,9) | |||
HbS/β°Thalassemia | 18 (21,2) | 8 (10,3) | |||
HbS/β+Thalassemia | 9 (10,5) | 10 (12,8) | |||
Haplotypes n (%) | |||||
Bantu/− | 79 (97,5) | 62 (93,9) | 0.41 | 0.45 | |
Bantu/Bantu | 48 (59,3) | 47 (71,2) | 0.14 | 0.18 | |
Benin/− | 27 (69,2) | 12 (18,2) | 2,25 [1,03-4,9] | 0.04 | 0.4 |
Benin/Benin | 1 (1,2) | 3 (4,5) | 0.33 | 0.5 | |
Alpha thalassemia n(%) | 56 (60,2) | 42 (58,3) | 0.87 | 0.8 | |
G6PD deficiency n(%) | |||||
G6PD-Deficiency | 5 (6,4) | 7 (10,3) | 0.02 | 0.2 | |
Heterozygote | 4 (5,1) | 12 (17,6) | |||
UGT1A1 mutation n (%) | 12 (14) | 11 (15,1) | 1 | 1 | |
SNP n (%) | |||||
BCL11A or rs4671393 | 20 (19,8) | 7 (8) | 3,13 [1,1-8,89} | 0.047 | 0.2 |
BCL11A rs11886868 | 28 (63,6) | 9 (29) | 4,28 [1,6-11,5] | 0.005 | 0.06 |
BCL11A rs1427407 | 28 (41,8) | 10 (15,2) | 4 [1,75-9,22] | 0.001 | 0.051 |
HMIP rs9399137 | 12 (14,3) | 2 (2,7) | 5,92 [1,28-27,4] | 0.01 | 0.24 |
Xmn1 or rs7842144 | 6 (7,2) | 4 (5,7) | 0.76 | 0.74 | |
BCL11A rs10189857 | 64 (79) | 55 (77,5) | 0.85 | 0.8 | |
HMIP rs28384513 | 30 (73,2) | 23 (74,2) | 1 | 1 | |
HMIP rs66650371 | 31 (41,3) | 22 (33,3) | 0.39 | 0.4 | |
HMIP rs4895441 | 7 (17,1) | 2 (6,5) | 0.28 | 0.3 | |
Hydroxyurea treatment | 11 (11) | 19 (22,1) | 0.047 | 0.5 | |
Osteonecrosis (n, %) | 1 (1,3) | 8 (11,1) | |||
Number of hospitalization (n, %) | 0.01 | 0.1 | |||
No | 21 (21) | 14 (16,5) | |||
1 à 2 per year | 77 (77) | 60 (70,6) | |||
≥ 3 per year | 2 (2) | 11 (12,9) | |||