TERMINOLOGIES Thrombosis - Formation of intravascular clots Petechiae - <3 mm hemorrhages into skin of different sizes Purpura - 1cm hemorrhages into skin of different sizes Ecchymosis - >3 cm hemorrhages into skin of different sizes Telangiectasia - Chronic dilation of the capillary walls Menorrhagia - Excessive menstrual bleeding Menorrhagia - Irregular uterine bleeding not related to menstruation Hematohidrosis - Sweating of blood Epistaxis - Nosebleed Hematemesis - Vomiting of blood Hemoptysis - Expectoration of blood Hemarthrosis - Leakage of blood into the joint cavity Hematochezia - Passage of fresh blood in stool Melena - Passage of old blood in stool VASCULAR DISORDERS The pathophysiology of disorders of vessels and their supporting tissues is obscure. Laboratory studies of platelets and blood coagulation usually yield normal results. The diagnosis is often based on medical history and is made by ruling out other sources of bleeding disorders. The usual clinical sign is the tendency to bruise easily or to bleed spontaneously, especially from mucosal surfaces. Hereditary Vascular Disorders · Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) · Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt 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 · Vitamin C deficiency (scurvy) Purpuras of Unknown Origin · Purpura simplex (easy bruisability) · Psychogenic purpura Hereditary Vascular Disorders Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) · Mode of inheritance is autosomal dominant · Telangiectasias (dilated superficial blood vessels that create small, focal red lesions) occur throughout the body but are most obvious on the face, lips, tongue, conjunctiva, nasal mucosa, fingers, toes, and trunk and under the tongue · Telangiectasias are fragile and prone to rupture · The lesions blanch when pressure is applied · Manifest by puberty . The diagnosis is based on the characteristic skin or mucous membrane lesions, a history of repeated hemorrhage, and a family history of a similar disorder Hemangioma-thrombocytopenia syndrome (Kasabach-Merritt syndrome) . External hemangiomas may become engorged with blood and resemble hematomas · The condition is present at birth Ehlers-Danlos syndrome . May be transmitted as an autosomal dominant, recessive, or X-linked trait · Manifested by hyperextensible skin, hypermobile joints, joint laxity, fragile tissues and bleeding tendency · Disorder generally can be ascribed to defects in collagen production, structure, or cross linking, with resulting inadequacy of the connective tissues Homocystinuria · This is a disorder of methionine metabolism, leading to an abnormal accumulation of homocysteine and its metabolites (homocystine, homocysteine- cysteine complex, and others) in blood and urine. · Increasing visual problems may lead to diagnosis of homocystinuria. Pseudoxanthoma Elasticum · A progressive disorder that is characterized by the accumulation of deposits of calcium and other minerals (mineralization) in elastic fibers . The symptoms: growth of yellowish bumps on the skin of the neck, under the arms, or in the groin area; reduced vision; periodic weakness in the legs (claudication); or bleeding in the gastrointestinal tract, particularly the stomach Osteogenesis Imperfecta · An autosomal dominant disorder that prevents the body from building strong bones. . People with Ol might have bones that break easily, which is why the condition is commonly called brittle bone disease. Marfan syndrome · Affects connective tissue - the fibers that support and anchor the organs and other structures in the body . Tall and thin with unusually long arms, legs, fingers and toes Acquired Vascular Disorders: Allergic purpura (Henoch-Schönlein purpura) · Primarily a disease of children, occurring most commonly in children 3 to 7 years of age. . It is relatively uncommon among individuals younger than age 2 and older than age 20. . Twice as many boys as girls are affected. · Result of allergic vasculitis which involves the skin, GIT, kidneys, heart and CNS . The appearance of the lesions may be very rapid and accompanied by itching . The lesions have been described as "palpable purpura" . Normal platelet count, bleeding time, blood coagulation . Elevated WBC and ESR Paraproteinemia · High concentrations of paraprotein can cause severe hemorrhagic manifestations as a result of a combination of hyperviscosity and platelet dysfunction · Platelet function abnormalities in IgA myeloma, Waldenstrom macroglobulinemeia, IgG myeloma · Poor correlation between abnormal results of protime, aptt, thrombin time, bleeding time and clinical bleeding · Treatment for the bleeding complications of these disorders is primarily reduction in the level of the paraprotein Amyloidosis · Amyloid is a fibrous protein consisting of rigid, linear, branching, aggregated fibrils approximately 7.5 to 10 nm wide and of indefinite length . Thrombosis and hemorrhage have been ascribed to amyloid deposition in the vascular wall and surrounding tissues. . Low factor X levels from binding of factor X to amyloid fibrils, hyperfibrinolysis related to excessive urokinase activity and platelet function alterations may enhance bleeding tendencies Senile purpura . Occurs more commonly in elderly men than in women and is due to a lack of collagen support for small blood vessels and loss of subcutaneous fat and elastic fibers · The dark blotches are flattened, are about 1 to 10 mm in diameter, do not blanch with pressure, and resolve slowly, often leaving a brown stain in the skin (age spots) . Laboratory tests are normal, and no other bleeding manifestations are present Drug-induced vascular purpuras · Purpura associated with drug-induced vasculitis occurs in the presence of functionally adequate platelets · Aspirin, warfarin, barbiturates, diuretics, digoxin, methyldopa, sulfonamides, iodides Vitamin C deficiency (scurvy) · Insufficient dietary intake of vitamin C (ascorbic acid) results in scurvy and decreased synthesis of collagen, with weakening of capillary walls and the appearance of purpuric lesions . Laboratory test results are invariably normal Purpuras of Unknown Origin Purpura simplex (easy bruisability) . Increased bruising that results from vascular fragility . The disorder usually affects women Psychogenic purpura · Also referred to as Gardner-Diamond syndrome, autoerythrocyte sensitization, or painful bruising syndrome . Rare and poorly understood clinical presentation in which patients develop unexplained painful bruises, mostly on the extremities and/or face, during times of stress Mechanical purpura Factitious purpura Schamberg's purpura