Drepanocytic syndromes

(see also : drepanocytosisthalassemias and hemoglobin C and  hemoglobin F)

Pathologies resulting from the association of sickle cell disease and another anomaly of the hemoglobin.


genotype

severity

HbA %

HbA2  %

HbF %

HbS %

frequency

AA (normal)


> 95

2,5-3,5

< 1

0


AS (drepanocytic trait)


60

2,5-3,5

< 1

40


SS

+++

0

2,5 -3,5

1-20

75-95

+++

SS/ α thal 1 ou α thal 2

+ à ++





++

S/β° thal

+++

0

4-6

1-20

70-90

+

S/ β+ thal

+ à ++

10-20

4-6

1-15

65-85

+

S/C

+ à ++

0

45-50

1-6

50

++

S/D Punjab

S/O  Arab

S/C Harlem

+++





rare

S/ PHHF (hereditary persistance of Hb F, see this term)

0 à +



95


rare

A/S Antilles

+





rare


The most severe sickle cell syndromes are: SS, SC, SThal, SO Arab, SD Punjab and SE.