Leg Ulcers in a Patient with Spherocytosis: A Clinicopathological Report

Vanscheidt, Wolfgang; Leder, O; Vanscheidt, E; Wokalek, H.; Wilmer, J; Hofmann, Elisabeth; Schöpf, Erwin

doi:10.1159/000247863

Cited by 14 publications

(6 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These inherited disorders of the red cell cytoskeleton lead to variable degrees of severity of haemolytic anaemia. Patients with these membranopathies have been reported to develop pulmonary thromboemboli (Hayag‐Barin et al , 1998), PH, and leg ulcers (Vanscheidt et al , 1990). It has been suggested that these complications are more frequent among patients that have undergone splenectomy (Schilling et al , 2008).…”

Section: Haemolytic Anaemias Associated With Pathological Vascular Comentioning

confidence: 99%

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Kato

Taylor

2010

Br J Haematol

View full text Add to dashboard Cite

Summary The breakdown of senescent or defective red blood cells releases red cell contents, especially hemoglobin, which scavenges nitric oxide (NO) and decomposes to heme and free iron. These are potent oxidants, all of which have promoted the evolution of inducible and vasculoprotective compensatory pathways to rapidly clear and detoxify hemoglobin, heme and iron. Chronic hemolytic red cell disorders as diverse as sickle cell disease, thalassemia, unstable hemoglobinopathy, cytoskeletal defects and enzymopathies have been linked to a clinical constellation of pulmonary hypertension, priapism, leg ulceration and possibly cerebrovascular disease and thrombosis. Besides free hemoglobin, hemolysis has been associated with extracellular arginase that limits substrate availability to NO synthase, endogenous inhibitors of NO synthase activity, and inappropriate activation of hemostatic pathways. This article reviews the hemolytic disorders that have been reported to manifest vascular complications, and explores the speculative possibility that hemolysis mediates some of the vascular complications of inflammation and diabetes.

show abstract

Section: Haemolytic Anaemias Associated With Pathological Vascular Comentioning

confidence: 99%

Pleiotropic effects of intravascular haemolysis on vascular homeostasis

Kato

Taylor

2010

Br J Haematol

View full text Add to dashboard Cite

show abstract

“…Leg ulcerations are a serious and debilitating complication of sickle cell disease (SCD) and other hemolytic anemias associated with markers of severe disease and early mortality, both in the United States and other countries . Their etiology and evolution is multifactorial and not completely characterized, but compromised blood supply secondary to vaso‐occlusion has been considered a determinant factor in their occurrence and delayed healing . Most recently endothelial dysfunction and thrombosis have been hypothesized to be contributing factors for this and other end‐organ damage in SCD .…”

Section: Introductionmentioning

confidence: 99%

Vasculopathy, inflammation, and blood flow in leg ulcers of patients with sickle cell anemia

et al. 2013

View full text Add to dashboard Cite

Chronic leg ulcers are frequent and debilitating complications of sickle cell anemia. Inadequate blood supply has been postulated to be an important factor in their occurrence and delayed healing. Little is known about their microcirculatory and histopathological changes. We evaluated the microcirculation of lower extremity ulcers with laser speckle contrast imaging and infrared thermography and obtained clinical and laboratory characteristics in 18 adults with sickle cell anemia and chronic leg ulcers. Skin biopsies were obtained in four subjects. Subjects had markers of severe disease, anemia, high degree of hemolysis, inflammation, and thrombophilia. The highest blood flow was present in the ulcer bed, progressively less in the immediate periwound area, and an unaffected control skin area in the same extremity. Microscopic examination showed evidence of venostasis, inflammation, and vasculopathy. Blood vessels were increased in number, had activated endothelium and evidence of thrombosis/recanalization. High blood flow may be due to chronic inflammation, cutaneous vasodilatation, venostasis, and in situ thrombosis. These changes in skin microcirculation are similar to chronic venous ulcers in the non-sickle cell disease (SCD) population, thus suggesting that leg ulcers may be another end-organ complication with endothelial dysfunction that appears in patients with SCD at a younger age and with higher frequency than in the general population.

show abstract

“…Such localized hemolysis has been shown within and outside of capillaries by electron microscopy in the vicinity of leg ulcers of a patient with spherocytosis. 10 Although 2% of patients with spherocytosis develop leg ulcers, 12 the clinical finding of recurrent pseudoerysipelas as seen in our patient has not been reported thus far. Because our patient had mild spherocytosis based on hematologic and serologic findings, 1 we hypothesize that his family carries an unknown mutation for a mild autosomal dominant phenotype.…”

Section: Discussionmentioning

confidence: 49%

“…1 The most frequently reported dermatologic complication of hereditary spherocytosis, reported in fewer than 2% of the patients, are leg ulcers at atypical locations, such as the back of the feet in patients withouth splenectomy. [10][11][12][13] Healing of refractory leg ulcers was reported after splenectomy. 11,14 The pathogenesis of the more commonly described leg ulcers in congenital spherocytosis is unknown.…”

Section: Discussionmentioning

confidence: 99%