Etiology and Pathogenesis Vascular Etiology The tumor microenvironment of many malignancies is rich with angiogenesis-stimulating growth factors; the most well-known is vascular endothelial growth factor (VEGF). The neoangiogenesis are typically unorganized and structurally dissimilar to the nonneoplastic vasculature and are…
Hereditary Vascular Malformations Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease) Hereditary hemorrhagic telangiectasia (HHT) is a systemic autosomal dominant (AD) vascular disease that is characterized by mucocutaneous telangiectasias and multiple arteriovenous malformations (AVMs). It is the most common inherited vascular bleeding disorder…
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). This disease can be divided into three major subtypes: typical HUS, atypical HUS (aHUS), and DEAP-HUS (deficiency…
Introduction Autoimmune lymphoproliferative syndrome (ALPS) was first described in 1967 when Canale and Smith reported on five children with massive lymphadenopathy and splenomegaly mimicking lymphoma. In 1992 Sneller et al. similarly described two patients with a progressive lymphoproliferative disorder associated with…