Vascular Disorders Flashcards
Hereditary vascular disorders
- Hereditary hemorrhagic Telangiectasia (Rendy-Osler-Weber Syndrome)
- Hemangioma-Thrombocytopenia Syndrome (Kasabach-Meritt Syndrome)
- Ehlers-Danlos Syndrome and Other Genetic Disorders
Acquired Vascular Disorders
- Allergic Purpura (Henoch-Schönlein Purpura)
- Paraproteinemia and Amyloidosis
- Senile Purpura
- Drug – Induced Vascular Purpuras
- Scurvy
Characterized by thin-walled blood vessels with discontinuous endothelium, inadequate smooth muscle, and inadequate / missing elastin in the surrounding stoma
Hereditary hemorrhagic Telangiectasia (Rendy-Osler-Weber Syndrome)
Universal finding of Hereditary hemorrhagic Telangiectasia (Rendy-Osler-
Weber Syndrome)
epistaxis (nosebleed)
dilated superficial blood vessels that create small, focal red lesions
Telangiectasia
Vascular tumor (Giant cavernous hemangioma), thrombocytopenia, bleeding diathesis
Hemangioma-Thrombocytopenia Syndrome (Kasabach-Meritt Syndrome)
Sequestration of platelets in the hemangiomas
Hemangioma-Thrombocytopenia Syndrome
(Kasabach-Meritt Syndrome)
May be transmitted as an autosomal dominant, recessive, or X-linked trait.
Ehlers-Danlos Syndrome
It is manifested by Hyperextensible skin, Hypermobile joints, Joint laxity
Ehlers-Danlos Syndrome
Be ascribed to defects in collagen production, structure, or cross-linking, with resulting inadequacy of the connective tissues
Ehlers-Danlos Syndrome
Generally applied to a group of nonthrombocytopenic purpura characterized by allergic manifestations including skin rash and edema
Allergic Purpura (Henoch-Schönlein Purpura)
More appropriately applied when the conditions an acute IgA-mediated disorder
Allergic Purpura (Henoch-Schönlein Purpura)
Implicates autoimmune microvascular injury
Allergic Purpura (Henoch-Schönlein Purpura)
Platelet function can be inhibiting by myeloma proteins
Paraproteinemia
IgA myeloma & IgG myeloma and Recurrent Purpurua
Dysproteinemia