The Spectra Optia device proved to be highly efficient in depleting RBCs in HC patients and allows for short procedure time. The Optia device can be safely used in this clinical setting. We recommend its use in case of severe iron overload if rapid iron depletion needs to be achieved and in case of cardiac compromise due to less blood volume removed.
SummaryBackgroundHereditary hemorrhagic telangiectasia is an inherited autosomal dominant disease presenting with recurrent bleeding episodes and iron deficiency anemia due to vascular malformations. Hereditary hemorrhagic telangiectasia is associated with an increased risk of stroke, gastrointestinal bleeding and pulmonary hypertension and life expectancy is significantly reduced. Excess vascular endothelial growth factor (VEGF) plays a key role in the pathophysiology of the disease.Case presentationHere we report about a male patient with hereditary hemorrhagic telangiectasia presenting with pulmonary and central nervous system involvement experiencing repetitive nosebleeds, necessitating frequent local cauterization and transfusion of more than 100 units of packed red blood cells. After initiation of temporary therapy with the anti-VEGF antibody bevacizumab at a dosage of 1 mg/kg body weight every 2 weeks, the nose bleeding episodes and the epistaxis severity score significantly decreased and long-lasting transfusion independence was achieved. Reinitiation of low-dose bevacizumab after relapse again proved effective without any documented therapy-related adverse events. In comparison to other reported anti-VEGF antibody protocols in hereditary hemorrhagic telangiectasia, our treatment approach proved to be cost-efficient.ConclusionIntermittent low-dose therapy with bevacizumab represents an effective and cost-efficient treatment option for transfusion-dependent patients with hereditary hemorrhagic telangiectasia.
Early causal therapy with treatment based on the stage of the wound, consequent goal-oriented interdisciplinary care, and relapse prophylaxis is critical for successful healing of venous ulcers. The costs of treatment significantly correlate with the duration of treatment which can be reduced by up to 60% using guideline-based concepts for the treatment of chronic wounds. Treatment success, in particular with regard to cost-benefit considerations, can be optimized by telemedicine networks of key players who treat chronic wounds.
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