thrombotic thrombocytopenic purpura

purpura /pur·pu·ra/ (pur´pu-rah)

1. a small hemorrhage in the skin, mucous membrane, or serosal surface.

2. a group of disorders characterized by the presence of purpuric lesions, ecchymoses, and a tendency to bruise easily.purpu´ric

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allergic purpura , anaphylactoid purpura Henoch-Schönlein p.

purpura annula´ris telangiecto´des  a rare form in which punctate erythematous lesions coalesce to form an annular or serpiginous pattern.

fibrinolytic purpura  purpura associated with increased fibrinolytic activity of the blood.

purpura ful´minans  nonthrombocytopenic purpura seen mainly in children, usually after an infectious disease, marked by fever, shock, anemia, and sudden, rapidly spreading symmetrical skin hemorrhages of the lower limbs, often associated with extensive intravascular thromboses and gangrene.

purpura hemorrha´gica  idiopathic thrombocytopenic p.

Henoch's purpura  Henoch-Schönlein purpura in which abdominal symptoms predominate.

Henoch-Schönlein purpura  nonthrombocytopenic purpura of unknown cause, usually in children; associated with symptoms such as urticaria, erythema, arthropathy and arthritis, gastrointestinal disorder, and renal involvement.

idiopathic thrombocytopenic purpura  thrombocytopenic purpura not directly associated with a systemic disease, although often following a systemic infection; believed to be due to an IgG immunoglobulin that acts as an antibody against platelets.

malignant purpura  meningococcal meningitis.

nonthrombocytopenic purpura  purpura without any decrease in the platelet count of the blood.

Schönlein purpura  Henoch-Schönlein purpura with articular symptoms and without gastrointestinal symptoms.

Schönlein-Henoch purpura  Henoch-Schönlein p.

purpura seni´lis  dark purplish red ecchymoses occurring on the forearms and backs of the hands in the elderly.

thrombocytopenic purpura  any form in which the platelet count is decreased, occurring as a primary disease (idiopathic thrombocytopenic p.) or as a consequence of a primary hematologic disorder (secondary thrombocytopenic p.) .

thrombotic thrombocytopenic purpura  a form of thrombotic microangiopathy marked by thrombocytopenia, hemolytic anemia, neurological manifestations, azotemia, fever, and thromboses in terminal arterioles and capillaries.

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thrombotic thrombocytopenic purpura

n.

A disease of unknown origin, characterized by abnormally low levels of platelets in the blood, the formation of blood clots in the arterioles and capillaries of many organs, and neurological damage.

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thrombotic thrombocytopenic purpura (TTP)

Etymology: Gk, thrombos, lump, thrombos + kytos, cell, penia, poverty; L, purpura, purple

a disorder characterized by thrombocytopenia, hemolytic anemia, and neurologic abnormalities. It is accompanied by a generalized purpura with the deposition of microthrombi within the capillaries and smaller arterioles. It includes a chronic form and an acute fulminating form that may be fatal in weeks. Therapy includes corticosteroids, splenectomy, and therapeutic plasma exchange. Compare disseminated intravascular coagulation. See also thrombocytopenic purpura.

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purpura (pur´pyoor),

n an extravasation of blood into the tissues, resulting in blue to black lesions of the skin or mucosa (petechiae and ecchymoses).

Purpura.

purpura, allergic,

n (anaphylactoid purpura) a thrombocytopenic or nonthrombocytopenic purpura related to an allergic reaction. Manifestations include the common symptoms of allergy.

purpura, anaphylactoid

n See purpura, allergic.

purpura, essential,

n See purpura, thrombocytopenic, idiopathic.

purpura hemorrhagica

n See purpura, thrombocytopenic and purpura, thrombocytopenic, idiopathic.

purpura, idiopathic thrombocytopenic,

n (essential purpura, land scurvy, primary purpura, purpura hemorrhagica) a type of unknown cause.

purpura, nonthrombocytopenic,

n a type related to increased capillary permeability. Included are allergic purpuras and those resulting from vitamin C deficiency, bacterial toxins (scarlet fever, typhoid), drug intoxications, and metabolic toxins (nephritis, liver disease).

purpura, primary,

n See purpura, thrombocytopenic, idiopathic.

purpura, secondary,

n See purpura, thrombocytopenic, symptomatic.

purpura, symptomatic thrombocytopenic,

n (secondary purpura) a type resulting from the effects of chemical, physical, vegetable, or animal agents or infections or related blood disorders.

purpura, thrombocytopathic

n a type associated with qualitative abnormalities of the platelets.

purpura, thrombocytopenic

n (essential thrombopenia, pseudohemophilia, hemorrhagica, Werlhof's disease) a type characterized by severe ecchymoses and petechiae associated with marked reduction in the numbers of blood platelets. There is prolonged bleeding time and poor clot retraction, but the coagulation and prothrombin times are normal. Hemorrhage may occur spontaneously from any area of the oral mucosa. This disease may be acute and fatal, whereas in other instances it may run a chronic course with intermittent attacks.

purpura, thrombotic thrombocytopenic,

n a febrile disease of unknown cause characterized by hemolytic anemia, neurologic symptoms, hemorrhage into the skin and mucous membranes, icterus, hepatosplenomegaly, low platelet count, and platelet thrombi occluding capillaries and arterioles.

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thrombotic thrombocytopenic purpura

Moschcowitz's disease; TTP Hematology A rare–1:10⁶/yr disorder of the microcirculation, most common in ♀ age 20-50 Clinical Moschcowitz's pentad: Thrombocytopenia, splenomegaly, DIC, fever, neurologic signs–eg, headache, confusion, aphasia, transient paresis, ataxia, sensory defects and coma, as well as pallor, jaundice, fibrin thrombi in renal vessels, heart, liver and spleen, epistaxis, cerebral, retinal, vaginal hemorrhage, heart dysfunction, hepatomegaly, pancreatitis Associations SLE, rheumatoid arthritis, Sjögren syndrome Lab ↓ Platelets, usually < 50,000/mm³, normal coagulation factors, ↓ complement proteins, Coombs-negative microangiopathic hemolytic anemia–often severe, 30% have ↓ Hb < 55g/L, US: < 5.5 g/dl and reticulocytosis, schistocytes, burr cells, helmet-shaped RBCs, normoblasts, reticulocytosis, ↑ unconjugated BR, ↑ Hb, ↑ hemosiderin, ↓ haptoglobin, proteinuria Management Corticosteroids, aspirin, dipyridamole, Igs; if no response, plasmapheresis. See Hemolytic-uremic syndrome.