Wound healing in hemoglobinopathy

Leg ulcers are the most common cutaneous complication of sickle cell disease. These lesions occur mainly in homozygous forms, are slow to heal and often relapse, causing negative physical, emotional, and economic impacts. In this paper, we discuss the clinical presentation, diagnosis, and pathophysiology of sickle cell leg ulcers and their implications for treatment.

OBJECTIVE: Thalassemia is a heterogeneous group of congenital hemoglobinopathies caused by mutations in the globin gene complex that result in an unbalanced globin synthesis. Unmatched globin chains bind to the cytosolic surface of red blood cell membrane where they cause oxidative damage that might in part be responsible for membrane weakness. The deformability of red blood cells and hypercoagulable state in thalassemic patients have been incriminated in leg ulcer formation, as this might cause ischemia to the skin and consequently friability and ulceration...

Major hemoglobinopathies, including sickle cell anemia, are becoming a global health issue. Leg ulcers are the most common cutaneous manifestation of sickle cell disease and an important contributor to morbidity burden in this population. Leg ulcers following sickling disorders are extremely painful, and hard to heal. The clinical evidence for the optimal management of these ulcers is limited. Treating the cause and the strategies to prevent sickling are the mainstay of treatment. The basic principles of wound bed preparation and compression therapy is beneficial in these patients...

Chronic cutaneous ulcers are commonplace in the developing world, especially in rural areas with poor living conditions and often result from the trauma of road-traffic injuries. Chronic cutaneous ulcers may also be due to vascular insufficiency, neuropathy, nodular leprosy, pressure, diabetes, or hemoglobinopathies, or they may be tropical ulcers. If poorly managed, these lesions may undergo malignant transformation. We evaluated the clinical histories and treatment outcomes of patients seen at the University of Calabar Teaching Hospital, between January 2000 and December 2004, who had histologic diagnosis of Marjolin's ulcer, in an attempt to identify risk factors for this problem...

Leg ulceration is a complication associated with sickle cell disorders. Caroline Dowsett outlines why this problem occurs and describes the management of a patient with sickle cell disorder who presented with an infected leg ulcer. She concludes that there is a need for more research into the management of these complex wounds.

METHODS: Over a period of 11 y, 50 patients (22 males, 28 females; age range, 8 mo to 22 y) presenting with sickle cell-associated bone pain underwent 93 sequential examinations with 99mTc-sulfur colloid bone marrow scanning and 99mTc-diphosphonate bone scanning. Multiple examinations were performed on 21 patients. The number and distribution of total acute, healed, and nonhealed infarcts by location were recorded on a skeletal homunculus. RESULTS: For this population, the total number of sites of bone and bone marrow infarction was 464...

The fetus can now be considered a patient. Sophisticated imaging techniques and prenatal tests have allowed precise diagnosis of fetal abnormalities. Subsequent recommendations for treatment are evolving depending on diagnosis and progression of disease. For severely affected fetuses with life-threatening problems, fetal surgery is a proposed intervention. Knowledge of the diagnosis and potential treatment modalities are now a prerequisite for providing the necessary support and care of mothers of fetuses with an anomaly...