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Encyclopedia > Idiopathic thrombocytopenic purpura
Idiopathic thrombocytopenic purpura
Classification & external resources
ICD-10 D69.3
ICD-9 287.31
OMIM 188030
DiseasesDB 6673
eMedicine emerg/282 
MeSH D016553

Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count (thrombocytopenia) of no known cause (idiopathic). As most causes appear to be related to antibodies against platelets, it is also known as immune thrombocytopenic purpura. Although most cases are asymptomatic, very low platelet counts can lead to a bleeding diathesis and purpura. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... A 250 ml bag of newly collected platelets. ... Thrombocytopenia (or -paenia, or thrombopenia in short) is the presence of relatively few platelets in blood. ... Each antibody binds to a specific antigen; an interaction similar to a lock and key. ... In medicine, a disease is asymptomatic when it is at a stage where the patient does not experience symptoms. ... In medicine (hematology), a bleeding diathesis is a propensity to bleeding (hemorrhage) due to a defect in the system of coagulation. ... Purple discolorations on the skin caused by bleeding underneath the skin. ...

Contents

Signs and symptoms

ITP occurs most often in women over 40 years of age with the male female ratio about 1:1.5. It may be acute, lasting for 6 months or less, or chronic, lasting for over a year. The acute type is more often seen in children and will cure itself in more than 80% of cases. The chronic type is more commonly seen in adults and it tends to get worse as the disease progresses.


Occasionally, ITP patients suffer from petechiae, bruising, nosebleeds, and bleeding gums; this is the characteristic pattern of bleeding in platelet disorders. Bleeding normally does not occur unless the platelet count is very low (below about 10,000 per mm3, compared to a normal range of 150,000–400,000 per mm3). minor Petechia A petechia (IPA pronunciation: ), plural petechiae (IPA pronunciation: ) is a small red or purple spot on the body, caused by a minor hemorrhage (broken capillary blood vessels). ... A bruise or contusion or ecchymoses is a kind of injury, usually caused by blunt impact, in which the capillaries are damaged, allowing blood to seep into the surrounding tissue. ... A nosebleed or nosebleedage, medically known as epistaxis, is the relatively common occurrence of hemorrhage (bleeding) from the nose, usually noticed when it drains out through the nostrils. ... The gingiva, or gums, consist of the tissue surrounding the roots of the teeth and covering the jawbone. ...


Subarachnoid and intracerebral hemorrhage are very serious possible complications of this disease. Fortunately, these are rare in patients who are being treated. A subarachnoid hemorrhage (SAH) is bleeding into the subarachnoid space surrounding the brain, i. ... ...


Pathogenesis

In many cases, the cause is not actually idiopathic but autoimmune, with antibodies against platelets being detected in approximately 60% of patients. Most often these antibodies are against platelet membrane glycoproteins IIb-IIIa or Ib-IX, and are of the IgG type. The coating of platelets with IgG renders them susceptible to opsonization and phagocytosis by splenic macrophages. Idiopathic means arising spontaneously or from an obscure or unknown cause. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Wikipedia does not yet have an article with this exact name. ... A glycoprotein is a macromolecule composed of a protein and a carbohydrate (an oligosaccharide). ... Schematic of antibody binding to an antigen An antibody is a protein complex used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ... An opsonin is any molecule that acts as a binding enhancer for the process of phagocytosis. ... Phagocytosis is a form of endocytosis wherein large particles are enveloped by the cell membrane of a (usually larger) cell and internalized to form a phagosome, or food vacuole. ... The spleen is an organ of the lower abdomen, where it functions in the destruction of old red blood cells and holding a reservoir of blood. ... A macrophage of a mouse stretching its arms to engulf two particles, possibly pathogens Macrophages (Greek: big eaters, makros = long, phagein = eat) are white blood cells, more specifically phagocytes, acting in the nonspecific defense as well as the specific defense system of vertebrate animals. ...


The IgG autoantibodies are also thought to damage megakaryocytes, the precursor cells to platelets, but this is thought to contribute only slightly to the decrease in platelet numbers.


Recent evidence suggests that the stimulus for autoantibody production in ITP is due to abnormal T helper cells reacting with platelet antigens on the surface of antigen presenting cells. This importamt finding suggests that therapies directed towards T cells may be effective in treating ITP.


Diagnosis

When measuring the platelet count, one has to bear in mind that the "normal values" for laboratory measures are all statistical. They are defined by the upper and lower 2.5th percentile. It is therefore possible to be completely healthy but to have a decreased platelet count. There is, however, a higher chance of pathology.


The diagnosis of ITP is a clinical one and is a diagnosis of exclusion. Low platelet count can be a feature of a large number of diseases and, when serious, warrants investigation by a hematologist. Secondary causes include leukemia, medications (e.g. quinine, heparin), lupus erythematosus and some other autoimmune disorders, cirrhosis (leading to thrombocytopenia from hypersplenism), HIV, congenital causes, and antiphospholipid syndrome. A bone marrow examination may be performed on patients over the age of 60 and people who do not respond to treatment, or when the diagnosis is in doubt. Hematology is the branch of medicine that is concerned with blood and its disorders. ... Leukemia or leukaemia (see spelling differences) is a cancer of the blood or bone marrow and is characterized by an abnormal proliferation (production by multiplication) of blood cells, usually white blood cells (leukocytes). ... Quinine (IPA: ) is a natural white crystalline alkaloid having antipyretic, anti-malarial with analgesic and anti-inflammatory properties and a bitter taste. ... Heparin is a highly sulfated glycosaminoglycan widely used as an injectable anticoagulant. ... Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ... Cirrhosis is a consequence of chronic liver disease characterized by replacement of liver tissue by fibrotic scar tissue as well as regenerative nodules, leading to progressive loss of liver function. ... Species Human immunodeficiency virus 1 Human immunodeficiency virus 2 Human immunodeficiency virus (HIV) is a retrovirus that causes acquired immunodeficiency syndrome (AIDS, a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections). ... This article, image, template or category should belong in one or more categories. ... A bone marrow biopsy is a medical procedure used as part of a test in the diagnosis of several conditions including leukemia. ...


Despite the destruction of platelets by splenic macrophages, the spleen is normally not enlarged. In fact, an enlarged spleen should lead a clinician to investigate other possible causes for the thrombocytopenia.


Accelerated formation of platelets results in the presence of abnormally large platelets which are seen in a peripheral blood smear. Overall bleeding time is prolonged in these patients, but prothrombin time (PT) and partial thromboplastin time (PTT) are normal (because the problem is with the platelets, not with the coagulation cascade). A microscopic view of an abnormal blood film. ... The prothrombin time (PT) and its derived measures of prothrombin ratio (PR) and international normalized ratio (INR) are measures of the extrinsic pathway of coagulation. ... The partial thromboplastin time (PTT) or activated partial thromboplastin time (aPTT or APTT) is a performance indicator measuring the efficacy of both the intrinsic and the common coagulation pathways. ... The coagulation cascade The coagulation cascade. ...


Occasionally, autoimmune hemolytic anemia and immune thrombocytic purpura may coexist, which is a condition called Evans syndrome. Evans Syndrome is an autoimmune disease in which an individuals antibodies attack their own RBCs as well as their platelets. ...


Treatment

Mild ITP does not require treatment. When platelet counts fall under 10,000 per microliter, or under 50,000 when hemorrhage occurs (e.g. in the digestive tract or in a severe nosebleed) treatment is generally initiated with steroids. Intravenous immunoglobulin (IVIg) is often used initially in order to raise the count quickly. It is also a common treatment for children. When these strategies fail, splenectomy (removal of the spleen) is sometimes undertaken, as platelets targeted for destruction will often meet their fate in the spleen. Splenectomy is said to be successful in 60% of cases although it is less successful in older people. A relatively new strategy is treatment with anti-D, an agent used in mothers who have been sensitized to rhesus antigen by a Rh+ baby, but the patient must be Rh+. Immunosuppresants like mycophenolate mofetil and azathioprine are becoming more popular for their effectiveness. Rituximab has also been used with some success for some patients. A 250 ml bag of newly collected platelets. ... This article is in need of attention. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... For the Physics term GUT, please refer to Grand unification theory The gastrointestinal or digestive tract, also referred to as the GI tract or the alimentary canal or the gut, is the system of organs within multicellular animals which takes in food, digests it to extract energy and nutrients, and... A nosebleed or nosebleedage, medically known as epistaxis, is the relatively common occurrence of hemorrhage (bleeding) from the nose, usually noticed when it drains out through the nostrils. ... Steroid skeleton of lanosterol. ... Intravenous immunoglobulin (IVIG) is a blood product administered intravenously. ... A splenectomy is a procedure that involves the removal of the spleen by operative means. ... In Greek mythology, King Rhesus of Thrace fought for Troy during the Trojan War. ... Mycophenolic acid (INN) (IPA: ) or mycophenolate is an immunosuppressant drug used to prevent rejection in organ transplantation. ... Azathioprine is a chemotherapy drug, now rarely used for chemotherapy but more for immunosuppression in organ transplantation, autoimmune disease such as rheumatoid arthritis or inflammatory bowel disease such as Crohns disease. ... Rituximab, sold under the trade names Rituxan® and MabThera®, is a monoclonal antibody used in the treatment of B cell non-Hodgkins lymphoma, B cell leukemia, and some autoimmune disorders. ...


Extreme cases (very rare, especially rare in children) may require vincristine, a chemotherapy agent, to stop the immune system from destroying platelets. Vincristine (Oncovin®) is a vinca alkaloid from the Madagascar periwinkle (Catharanthus roseus, formerly Vinca rosea and hence its name). ... Chemotherapy is the use of chemical substances to treat disease. ...


Intravenous immunoglobulin, while sometimes effective although not all patients respond, is expensive and the improvement is temporary (generally lasting less than a month). However, in the case of a pre-splenectomy ITP patient with dangerously low platelet counts, and a poor response to other treatments, IVIg treatment can increase platelet counts, making the splenectomy operation less dangerous. It is also commonly used as a long-term (though monthly) treatment. Intravenous immunoglobulin (IVIG) is a blood product administered intravenously. ...


Platelet transfusion is not normally recommended and is usually unsuccessful in raising a patient's platelet count. This is because the underlying autoimmune mechanism that destroyed the patient's platelets to begin with will also destroy donor platelets. An exception to this rule is when a patient is bleeding profusely, when transfusion of platelets can quickly form a platelet plug to stop bleeding.


AMG 531 is an experimental treatment for stimulating platelet production. Initial clinical trials show it to be moderately effective in chronic ITP,[1] but its role in the treatment of ITP is still unknown, given that existing treatments are generally effective and that the underlying problem in ITP is the increased destruction of platelets, not their production.


Synonyms

ITP knows many synonyms, but idiopathic or immunological thrombocytopenic purpura are the most common names. There's also an eponym, Werlhof's disease, but this is used infrequently. An eponym is the name of a person, whether real or fictitious, who has (or is thought to have) given rise to the name of a particular place, tribe, discovery, or other item. ...


Other synonyms include: essential thrombocytopenia, haemogenia, haemogenic syndrome, haemorrhagic purpura, idiopathic thrombopenic purpura, morbus haemorrhagicus maculosus, morbus maculosis haemorrhagicus, morbus maculosus werlhofii, peliosis werlhofi, primary splenic thrombocytopenia, primary thrombocytopenia, primary thrombocytopenic purpura, purpura haemorrhagica, purpura thrombocytopenica, purpura werlhofii, splenic thrombocytopenic purpura, thrombocytolytic purpura.


References

  • Cines DB, Blanchette VS (2002). "Immune thrombocytopenic purpura". N Engl J Med 346 (13): 995–1008. PMID 11919310.
  • Cines D, Bussel J (2005). "How I treat idiopathic thrombocytopenic purpura (ITP)". Blood 106 (7): 2244–51. PMID 15941913.  Review article, free full text
  • Provan D, Newland A (2002). "Fifty years of idiopathic thrombocytopenic purpura (ITP): management of refractory ITP in adults". Br J Haematol 118 (4): 933–44. PMID 2199770.  Free fulltext.
  • Coopamah M, Garvey M, Freedman J, Semple J (2003). "Cellular immune mechanisms in autoimmune thrombocytopenic purpura: An update". Transfus Med Rev 17 (1): 69–80. PMID 12522773. 
  • Semple JW and J Freedman. Enhanced anti platelet T lymphocyte reactivity in patients with autoimmune thrombocytopenia (ATP). Blood. 78(10):2619-2625, 1991.
  • ITP Primer: ITP (idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura). MediPrimer.com. Retrieved on 2007-02-14.
  • Kumar, Vinay, Abul Abbas, and Nelson Fausto. Robins and Cotran Pathologic Basis of Disease, 7th ed. (2004). ISBN 81-8147-528-3
  • Sacher, Ronald A. and Richard A. McPherson. Wildman's Clinical Interpretation of Laboratory Tests, 11th ed. (2000). ISBN 0-8036-0270-7
  • Drug Induced Thrombocytopenia. Platelet Disorder Support Association. Retrieved on 2007-02-14.
  • synd/3349 at Who Named It - Paul Gottlieb Werlhof

  Results from FactBites:
 
Idiopathic thrombocytopenic purpura - MayoClinic.com (294 words)
Idiopathic thrombocytopenic purpura (ITP), also called immune thrombocytopenic purpura, is a bleeding disorder in which your blood has difficulty clotting due to an unusually low number of platelets.
People with idiopathic thrombocytopenic purpura, because of their low platelet count, tend to bruise easily and bleed longer when injured.
Idiopathic thrombocytopenic purpura is often divided into two categories: acute and chronic.
  More results at FactBites »

 
 

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