HEMA

• Hereditary Hemorrhagic Telangiectasia is also known as Osler-Weber-Rendu Syndrome
• HHT is characterized as the presence of widespread telangiectatic lesions of the skin and mucous membrane
• BT, PFT, Tourniquet test and Coagulation test are normal in HHT
• Iron Deficiency Anemia is usual
• Hemangioma Thrombocytopenia Syndrome or Kasabach-Merritt Syndrome is characterized by a profound thrombocytopenia related to platelet trapping within a vascular tumor, either a Kaposi-like hemangioendothelioma or a tufted angioma.
• In Kasabach-Merritt Syndrome, thrombocytopenia is usually severe and associated with Disseminated Intravascular Coagulation (DIC) and Microangiopathic Hemolytic Anemia (MAHA).
• Platelet trapping demonstrated by immunohistochemical staining of the tumors with anti-CD61 antibodies.
• Ehlers-Danlos Syndrome is characterized by hyper distensible joints and fragile skin.
• In Ehlers-Danlos Syndrome, physical findings are large skin ecchymosis and hematomas, bleeding from the gums, and GIT bleeding.
• Marfan Syndrome is a genetic disorder due to mutation of the gene for fibrillin resulting in abnormalities of connective tissues and risk for bleeding and bruising.
• Pseudoxanthoma Elasticum is an autosomal recessive disorder affecting elastic fibers of connective tissue of skin and arteries.
• PXE is caused by autosomal recessive mutations in the ABC6 gene on the short arm of chromosome 16.
• Osteogenesis Imperfecta is a rare autosomal disorder caused by mutation of genes which code for peptides of type I collagen.
• Purpura refers to visible hemorrhage into mucous membrane or skin.
• Allergic Purpuras/Anaphylactoid Purpura is characterized by a relatively distinctive purpuric eruption. Also the result of an autoimmune process or allergic vasculitis.
• Nonthrombocytopenic purpura is characterized by allergic manifestation (skin rash and edema)
• Henoch-Schönlein Purpura is a result of allergic vasculitis which involves the skin, GIT, kidneys, heart and CNS.
• Henoch-Schönlein Purpura is commonly seen in children; renal dysfunction is common and typically reversible in children.
• Septic means infected with bacteria
• An embolus is anything that moves through blood vessels until it gets stuck in a vessel that is too small to pass through and stops the blood flow
• Septic emboli are bacteria containing blood clots that have broken free of their source and traveled through the bloodstream until getting lodged in and blocking a blood vessel.
• Pupura Fulminans is any purpura on rapid onset
• Major initiating factor in Purpura Fulminans appears to be diffused vascular injury and intra-vascular coagulation.
• Waldenstrom's Purpura is a disorder of women that presents with recurrent purpura on the lower extremities and resultant hemosiderin staining of the skin similar to Schamberg's.
• Cryoglobulinemia is caused by production of cryoprecipitable serum proteins or protein complexes or from hepatitis C.
• Hyperviscosity Syndrome results from hypergammaglobulinemia owing to an increase in plasma viscosity.
• The clinical representation of hyperviscosity syndrome consists principally of the triad of mucosal bleeding, visual changes, and neurologic symptoms.
• Amyloidosis is characterized by low factor X levels from binding of Factor X to amyloid fibrils.
• Senile Purpura is seen in older individuals; or in individuals undergoing corticosteroid therapy. Purpuric lesions occur on the hand and arms.
• Drug induced purpura is caused by iodides, quinine, procaine, penicillin, and aspirin.
• Scurvy is caused by a deficiency in Vitamin C and decreased synthesis of collagen with weakening of capillary walls.
• Purpura simplex is simple purpura, characterized by Devil's pinches.
• Psychogenic purpura is seen in individuals with emotional problems, often after severe trauma or extensive surgery
• Atherosclerosis


  Build up fat in the blood vessel