thrombotic thrombocytopenic purpura

(redirected from Familial Thrombotic Thrombocytopenia Purpura)
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purpura

 [per´pu-rah]
a hemorrhagic disease characterized by extravasation of blood into the tissues, under the skin, and through the mucous membranes, and producing spontaneous bruises, ecchymoses, and petechiae (small hemorrhagic spots) on the skin. (See plate in Dermatology Atlas.) When accompanied by a decrease in the circulating platelets, it is called thrombocytopenic purpura; when there is no decrease in the platelet count, it is called nonthrombocytopenic purpura. adj., adj purpu´ric.

There are two general types of thrombocytopenic purpura: primary or idiopathic, in which the cause is unknown, and secondary or symptomatic, which may be associated with exposure to drugs or other chemical agents, systemic diseases such as multiple myeloma and leukemia, diseases affecting the bone marrow or spleen, and infectious diseases such as rubella (German measles).
Symptoms. The outward manifestations and laboratory findings of primary and secondary thrombocytopenic purpura are similar. There is evidence of bleeding under the skin, with easy bruising and the development of petechiae. In the acute form there may be bleeding from any of the body orifices, such as hematuria, nosebleed, vaginal bleeding, and bleeding gums. The platelet count is below 100,000 per cubic millimeter of blood and may go as low as 10,000 per cubic millimeter (normal count is about 250,000 per cubic millimeter). The bleeding time is prolonged and clot retraction is poor. Coagulation time is normal.
Treatment. Differential diagnosis is necessary to determine the type of purpura present and to eliminate the cause if it can be determined. General measures include protection of the patient from trauma, elective surgery, and tooth extractions, any one of which may lead to severe or even fatal hemorrhage. In the thrombocytopenic form, corticosteroids may be administered when the purpura is moderately severe and of short duration. Splenectomy is indicated when other, more conservative measures fail and is successful in a majority of cases. In some instances, especially in children, there may be spontaneous and permanent recovery from idiopathic purpura. (See Atlas 2, Part Q.)
allergic purpura (anaphylactoid purpura) Henoch-Schönlein purpura.
annular telangiectatic purpura a rare form in which pinpoint reddened lesions coalesce to form a ring-shaped or continuously linked pattern, commonly beginning in the lower extremities and becoming generalized. Called also Majocchi's disease.
fibrinolytic purpura purpura associated with increased fibrinolytic activity of the blood.
purpura ful´minans a form of 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 primary thrombocytopenic purpura.
Henoch's purpura a type of Henoch-Schönlein purpura in which abdominal symptoms predominate.
Henoch-Schönlein purpura a type of nonthrombocytopenic purpura, of unknown cause but thought to be due to a vasculitis; it is most often seen in children and is associated with clinical symptoms such as urticaria and erythema, arthritis and other joint diseases, gastrointestinal symptoms, and renal involvement. Called also Schönlein-Henoch purpura or syndrome and allergic or anaphylactoid purpura.
idiopathic thrombocytopenic purpura thrombocytopenic purpura not directly associated with any definable systemic disease, although it often follows a systemic infection; the cause is thought to be an IgG immunoglobulin that acts as an antibody against platelets, causing ecchymoses, petechiae, and other bleeding. There are both acute and chronic forms: the acute form has a sudden onset, is more common in children, and usually resolves spontaneously within a few months; the chronic form has a slower onset, is more common in adults, and may be recurrent.
nonthrombocytopenic purpura purpura without any decrease in the platelet count of the blood. In such cases the cause is either abnormal capillary fragility or a clotting factor deficiency.
Schönlein purpura a type of Henoch-Schönlein purpura in which articular systems predominate; called also Schönlein's disease.
Schönlein-Henoch purpura Henoch-Schönlein purpura.
purpura seni´lis dark purplish red ecchymoses occurring on the forearms and backs of the hands in the elderly.
thrombocytopenic purpura purpura associated with a decrease in the number of platelets in the blood; see purpura.
thrombotic thrombocytopenic purpura a form of thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, bizarre neurological manifestations, uremia (azotemia), fever, and thromboses in terminal arterioles and capillaries; some authorities consider it identical to the hemolytic uremic syndrome. Called also microangiopathic hemolytic anemia and Moschcowitz's disease.

throm·bot·ic throm·bo·cy·to·pe·nic pur·pu·ra

a rapidly fatal or occasionally protracted disease with varied symptoms in addition to purpura, including signs of central nervous system involvement, due to formation of fibrin or platelet thrombi in arterioles and capillaries in many organs.

thrombotic thrombocytopenic purpura (TTP)

Etymology: Gk, thrombos, lump, thrombos + kytos, cell, penia, poverty; L, purpura, purple
a disorder characterized by thrombocytopenia, hemolytic anemia, and neurological 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.

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.

throm·bot·ic throm·bo·cy·to·pe·nic pur·pu·ra

(throm-bot'ik throm'bō-sī'tō-pē'nik pŭr'pyŭr-ă)
A rapidly fatal or occasionally protracted disease with varied symptoms in addition to dermal hemorrhage, including signs of central nervous system involvement, due to formation of fibrin or platelet thrombi in arterioles and capillaries in many organs.

thrombotic thrombocytopenic purpura

A disseminated thrombotic microangiopathy similar to the childhood haemolytic-uraemic syndrome and featuring low platelet counts, haemolytic anaemia, fever, kidney damage and neurological disorders. Affected people have unusually large forms of the von Willebrand factor, and it was reported at the end of 1998 that the non-familial form is due to an inhibitor of the protease enzyme that cleaves the von Willebrand factor. The familial form is due to a deficiency of the enzyme.

throm·bot·ic throm·bo·cy·to·pe·nic pur·pu·ra

(throm-bot'ik throm'bō-sī'tō-pē'nik pŭr'pyŭr-ă)
A rapidly fatal or occasionally protracted disease with varied symptoms in addition to dermal hemorrhage, including signs of central nervous system involvement, due to formation of fibrin or platelet thrombi in arterioles and capillaries in many organs.
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