Table 1. Clinical criteria and severity score based on phenotype.

Clinical criteria	Variables	Score (points)
Days of hospitalization/year	≤ 1	0
	2–7	2
	≥ 8	5
Severe vasoocclusive crisis/year	0	0
	1–2	2
	≥ 3	5
Blood transfusion/year	0	0
	1–2	2
	≥ 3	5
Hip disease	Absent	0
	Present	5
Leg ulcer	Absent	0
	Present	5
Hepatobiliary complications	Absent	0
	Cholecystectomy	2
	Present	5
Neurologic events	Absent	0
	Present	5
Renal disorders	Absent	0
	Present	5
BMI	19–27	0
	< 19	2
Total	≤ 5: ACP* (1)
	6–15: MCP** (2)
	≥ 16: SCP*** (3)

*ACP: Asymptomatic clinical phenotype;

**MCP: Moderate clinical phenotype;

***SCP: Severe clinical phenotype; BMI = Body Mass Index