Sickle cell leg ulcers: a frequently disabling complication and a marker of severity

Halabi-Tawil, M.; Lionnet, François; Girot, Robert; Bachmeyer, Claude; Levy, Paul; Aractingi, S.

doi:10.1111/j.1365-2133.2007.08323.x

Cited by 69 publications

(57 citation statements)

References 29 publications

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“…[27][28][29][30][31] In LaSHS, we found no differences in terms of the incidence of leg ulcers between patients who did or did not receive HU. However, it appears that the use of HU induces ulcers mainly in patients with previous history of SCD ulcers, suggesting that HU could act in conjunction with other vascular abnormalities.…”

Section: The Effect Of Hu On Chronic Complications Of Scdmentioning

confidence: 99%

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS)

Voskaridou

Christoulas

Bilalis

et al. 2010

Blood

386

359

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The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/␤ 0 -thal, and 165 with HbS/␤ ؉ -thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P ‫؍‬ .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/ HbS, HbS/␤ 0 -thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients. (Blood.

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Section: The Effect Of Hu On Chronic Complications Of Scdmentioning

confidence: 99%

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS)

Voskaridou

Christoulas

Bilalis

et al. 2010

Blood

386

359

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“…Sickle cell patients with leg ulcers constitute a relatively distinct sub-phenotype, characterized by less frequent hospitalizations for vaso-occlusive pain crisis [22,23], while they are prone to develop pulmonary hypertension [5,[24][25][26][27][28], suggesting that the two complications share a common pathophysiology. In a cohort of 20 SCD patients with leg ulcer who underwent right hearth catheterization for suspected pulmonary hypertension, 15 had confirmation of PAH (personal data, this cohort has been described in Mehari et al (2013) [24].…”

Section: Epidemiologymentioning

confidence: 99%

“…Exposure to air often intensifies the pain. Past medical history should include a careful documentation of previously attempted ulcer therapies, and special attention paid to complications that are reportedly more common in leg ulcer patients: pulmonary hypertension, stroke, priapism, acute chest syndrome [28,29], lower extremity venous thrombosis, and retinopathy (personal observation of a 22% incidence of retinopathy).…”

Section: Medical Historymentioning

confidence: 99%

Critical Reviews: How we treat sickle cell patients with leg ulcers

Minniti

Kato

2015

American J Hematol

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The past five decades have seen an improvement in the mortality and morbidity of sickle cell disease (SCD) because of prophylaxis against infectious complications, improved and expanded red cell transfusions, implementation of hydroxyurea therapy, and advances in supportive care. Now that the majority of patients in the western hemisphere reaches adulthood, end organ diseases are frequent, which include vasculopathic complications such as chronic leg ulcers. The management of patients with leg ulcers requires the hematologist to lead a team of health care professionals, and investigates the presence of associated, but potentially still occult signs of vasculopathy, such as pulmonary hypertension, renal disease, priapism and retinopathy. These complications may be asynchronous, and long term careful screening is indicated, in order to ensure early diagnosis and intervention. It is crucial to address both the immediate consequences of pain, infection and disability, and long term effects on quality of life, employment and stigma associated with chronic ulceration. Recent insights into their pathophysiology may have practical implications. We propose a holistic approach to the management of patients' physical and emotional problems and mechanisms of ulcers formation and delayed healing. An overview of topical and systemic therapies for chronic ulcers is given, with the understanding that wound care therapy is best left to the wound specialists, medical and surgical, with whom the hematologist must keep an open line of communication. In the absence of evidence-based guidelines, our opinion is based on both a critical review of the literature and our personal clinical and research experience. Am. J. Hematol. 91:22-30, 2016. V C 2015 Wiley Periodicals, Inc. Clinical CasesCase 1. A 49 year old African American woman with sickle cell anemia, bilateral hip replacements for avascular necrosis of the femoral head, pulmonary arterial hypertension documented by pulmonary artery catheterization, and history of laser treatment and vitrectomy for proliferative retinopathy and vitreous hemorrhage, presented with a very painful ulcer at the right malleolus. She has had three previous ulcers, on both legs, the first at age 32 years, attributed to excessive standing at her job, no trauma, which had responded to topical care and healed within few months of presentation. At that time she was not taking hydroxyurea, but has been taking it now for longer than 10 years with good fetal hemoglobin response: 17%. This current ulcer developed while she was experiencing high personal stress but no physical trauma. She rarely experiences vaso-occlusive pain crisis and has very infrequent hospitalizations. She was referred to the wound care service and after 7 months of unfruitful therapies, including surgical debridement, MIST ultrasound therapy, antibiotics, and compressions, she was started on monthly transfusions and her ulcer healed 5 months later. She has had consistently elevated serum lactate dehydrogenase (LDH) (>600 IU/L), serum dire...

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“…Other worldwide estimates differ. In a recently published study of 457 SCD patients followed for ten years in a French referral center, 5.5% of patients had leg ulcerations [11]. In contrast, Serjeant reported 75% of Jamaican SCD patients older than 30 years had either active or healed ulcers [12].…”

Section: Prevalencementioning

confidence: 99%

Cutaneous Manifestations of Sickle Cell Disease

Jones¹,

Blinder²,

Anadkat³

2013

OJBD

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Sickle cell leg ulcers: a frequently disabling complication and a marker of severity

Abstract: Leg ulcers are a major complication of SCD, given their severe consequences and frequent association with other specific organ damage, and they constitute in their 'prolonged' form a severely disabling condition that remains an important therapeutic challenge.

Cited by 69 publications

References 29 publications

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS)

The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS)

Critical Reviews: How we treat sickle cell patients with leg ulcers

Cutaneous Manifestations of Sickle Cell Disease

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