Chronic leg ulceration in homozygous sickle cell disease: the role of venous incompetence

Abstract

Summary. Chronic leg ulceration is a common cause of morbidity in Jamaican patients with homozygous sickle cell (SS) disease. Ulcers heal more rapidly on bed rest and deteriorate on prolonged standing, suggesting a role of venous hypertension in their persistence. This hypothesis has been tested by Doppler detection of venous competence in SS patients and in matched controls with a normal haemoglobin (AA) genotype in the Jamaican Cohort Study. Venous incompetence was significantly more frequent in SS disease [137/183 (75%)] than in non‐pregnant AA controls [53/137 (39%)]. Past or present ulceration occurred in 78 (43%) SS patients, with a highly significant association between leg ulceration and venous incompetence in the same leg (P < 0·001). Prominence and/or varicosities of the veins and spontaneous leg ulcers were more common among patients with multiple sites of incompetence. The association of venous incompetence with chronic leg ulceration identifies a further pathological mechanism contributing to the morbidity of SS disease. The cause of venous incompetence is unknown but the sluggish circulation associated with dependency, turbidity and impaired linear flow at venous valves, hypoxia‐induced sickling, the rheological effects of high white cell counts, and activation of components of the coagulation system may all contribute. Venous hypertension in SS patients with leg ulceration suggests that firm elastic supportive dressings might promote healing of chronic leg ulcers.

Venous incompetence, poverty and lactate dehydrogenase in Jamaica are important predictors of leg ulceration in sickle cell anaemia

“…The nature of ulcer onset was classified from the history as spontaneous, traumatic or unknown. Venous incompetence was determined as described by Clare et al (2002).…”

Venous-Like Leg Ulcers without Venous Insufficiency in Congenital Anemia: Successful Treatment Using Compression Bandages

“…Recalcitrant leg ulcers are a common complication of such conditions and may occur early in life. The incidence of leg ulcers in adults with sickle cell disease is reported to be approximately 30% 3 …”

Sickle cell leg ulcers: associations with haemolysis and SNPs in Klotho, TEK and genes of the TGF‐β/BMP pathway

“…Chronic venous ulcers have been associated with thrombophilia, perhaps because of the relationship between thrombophilia and venous thrombosis (Mackenzie et al , 2002). Whether or not venous pressure abnormalities contribute to the development of sickle cell leg ulcers is inconclusive (Billett et al , 1991; Mohan et al , 2000; Chalchal et al , 2001; Clare et al , 2002). We hypothesised that leg ulcers, similar to other complications of sickle cell disease, might be related to the severity of haemolytic anaemia and be effected by polymorphic genes that could modulate the subphenotypes of sickle cell disease (Adams et al , 1994; Ohene‐Frempong et al , 1998; Sarnaik & Ballas, 2001; Gladwin et al , 2004).…”