The pathogenesis of immune thrombocytopaenic purpura nichola cooper and james bussel department of pediatrics, weill medical college of cornell university, new york, ny, usa summary immune thrombocytopaenic purpura. If your thrombocytopenia is caused by an underlying condition or a medication, addressing that cause might cure it. In addition, it is now recognized that these antibodies may also impair platelet production, creating a dual cause of thrombocytopenia. Immune thrombocytopenia itp, also called idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura is an acquired thrombocytopenia caused by autoantibodies against platelet antigens. Idiopathic thrombocytopenic purpura itp is the autoimmunemediated destruction of platelets. Immune thrombocytopenia itp is an acquired thrombocytopenia, defined as a platelet count immune destruction of platelets. It is one of the more common causes of thrombocytopenia. Immune thrombocytopenic purpura itp, also known by the shorter name immune thrombocytopenia same abbreviation itp, can be understood by looking at the three terms that make up its name. Immune thrombocytopenia purpura itp, also known as idiopathic thrombocytopenic purpura, is a type of thrombocytopenic purpura defined as an isolated low platelet count with a normal bone marrow in. It is a condition in, which there is bruising purpura because there are fewer platelets in the blood than usual. Immune thrombocytopenia can also occur with other blood disorders, including a form of cancer of the bloodforming tissue known as chronic lymphocytic leukemia, and human immunodeficiency virus hiv infection.
Immune thrombocytopenic purpura, which may lead to bleeding, is typically caused by antibodies directed against the platelet glycoprotein iibiiia complex. Immune thrombocytopenia purpura in children generally resolves on its own and does not require any treatment in most cases. Our study shows that rituximab as secondline treatment for immune thrombocytopenic purpura does not significantly reduce the rate of longterm treatment failure. Idiopathic autoimmune thrombocytopenic purpura itp the most common cause of acute onset of thrombocytopenia in an otherwise well child estimated about 1 in 20,000 children a recent history of viral illness is described in 5065% of cases of childhood itp 1252015 idiopathic thrombocytopenic purpura. Immune thrombocytopenia itp diagnosis and treatment. Platelet and megakaryocyte disorders thrombocytopenia increased destruction or consumption of platelets published date. Itp is an autoimmune disease in which the immune system mistakes the platelets as being foreign and destroys them. Since the pathology behind immune thrombocytopenia is not completely understood, distinguishing a true autoimmunemediated thrombocytopenia from other pathologies can be difficult, and the presence of a true secondary itp may be hard to discern. It causes a characteristic red or purple bruiselike rash and an increased tendency to bleed. How i treat thrombotic thrombocytopenic purpura and. Cotrimoxazole induced acute thrombocytopenic purpura. Update on chronic immune thrombocytopenic purpura itp. There are multiple causes of platelet destruction including infection, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation or immune causes due to. The underlying disease process in childhood itp and adult itp may be fundamentally.
Our cancer specialists provide expert treatment for immune thrombocytopenic purpura itp, a blood disorder. The information contained herein is not intended nor implied to be a substitute for professional medical advice and is provided for educational purposes only. Itp has 2 distinct clinical syndromes, manifesting as an acute condition in children and a chronic condition in adults. Pdf idiopathic thrombocytopenic purpura and mmr vaccine.
In severe and symptomatic drug induced immune thrombocytopenia, treatment with systemic corticosteroids or high dose intravenous immunoglobulin may be effective in correcting the. Alemtuzumab treatment causes a rare and distinct form of secondary immune thrombocytopenic purpura itp, characterized by delayed onset, responsiveness to conventional therapies, and prolonged remission following treatment. Idiopathic thrombocytopenic purpura itp is a common hematologic disorder manifested by immune mediated thrombocytopenia. Jan 21, 2011 idiopathic thrombocytopenic purpura itp is a bleeding condition in which the blood doesnt clot as it should. Section 2 your childs diagnosis and treatment 1 idiopathic thrombocytopenia purpura itp what is idiopathic thrombocytopenia purpura or itp. Platelet count platelet count aitp the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Definition, diagnosis and treatment of immune thrombocytopenic purpura james n. Immune thrombocytopenic purpura itp is a blood disorder characterized by low platelets, the blood cells responsible for clotting. Immune thrombocytopenia itp is an acquired hemorrhagic condition characterized by the accelerated clearance of platelets caused by antiplatelet autoantibodies. Spleen size is normal in the absence of another underlying condition.
Idiopathic thrombocytopenic purpura is an autoimmune disease which involves opsonization of platelets by autoantibodies directed against different surface glycoproteins, leading to their premature. The cause of thrombotic thrombocytopenia purpura in most patients is an autoantibody that blocks the function of an protein known as adamts that helps to control blood clotting. Hypogammaglobulinemia may be part of several different immunological or malignant. Clinical updates in adult immune thrombocytopenia blood. For more information on this disorder, choose thrombotic thrombocytopenia purpura as your search term in the rare disease database. Immune thrombocytopenia is a medical term for an immune condition causing a shortage of platelets thrombocytopenia and bruising purpura. This is because platelets are being destroyed by the immune system. Immune thrombocytopenia national heart, lung, and blood. Immune thrombocytopenia develops in up to of patients with systemic lupus erythematosus sle which is associated with a broad array of autoantibodies. Typically, it is chronic in adults, but it is usually acute and selflimited in children. Symptoms can be mild, but for most patients the risk. Platelet disorder support association for people with itp.
Immune thrombocytopenia purpura itp, also known as idiopathic thrombocytopenic purpura, is a type of thrombocytopenic purpura defined as an isolated low platelet count with a normal bone marrow in the absence of other causes of low platelets. This is most common in young children 2 to 6 years old. Immune thrombocytopenic purpura itp is a bleeding disorder characterized by isolated thrombocytopenia platelet count immune thrombocytopenic purpura powerpoint presentation free to download id. This is due to a low number of blood cell fragments called platelets. Children usually have the acute shortterm type of itp.
Thrombotic thrombocytopenic purpura ttp and atypical haemolytic uraemic syndrome ahus are acute, rare life. With treatment, the chance of remission a symptom free. Treatment of immunemediated thrombocytopenia purpura with. Immune thrombocytopenia itp msd manual professional edition. Immune thrombocytopenic purpura itp is an autoimmune bleeding disorder associated with platelet destruction. Thrombotic thrombocytopenic purpura ttp is a rare condition but associated with 90% mortality if left untreated. Idiopathic thrombocytopenic purpura itp is an acquired hemorrhagic disorder characterized by. Rituximab as secondline treatment for adult immune. By ronale tucker rhodes, ms understanding idiopathic thrombocytopenic purpura t ry saying the words idiopathic thrombocytopenic purpura to someone. Treatment of immune thrombocytopenia purpura itp private.
Immune thrombocytopenic purpura itp harvard health. Pdf mmr vaccine and idiopathic thrombocytopenic purpura. Most adults with itp will eventually need treatment, as the condition often becomes severe or long term chronic. Management of immune thrombocytopenic purpura in adults. Treatment of immune mediated thrombocytopenia purpura with concurrent intravenous immunoglobulin and platelet transfusion. Immune thrombocytopenia purpura associated with multiple. Ppt immune thrombocytopenic purpura powerpoint presentation free to download id. Symptoms usually go away in less than 6 months often within a few weeks. The diagnosis remains one of exclusion, after other thrombocytopenic disorders are ruled out based on history, physical examination, and laboratory evaluation. It is one of the more common causes of thrombocytopenia in otherwise asymptomatic adults. Idiopathic thrombocytopenic purpura genetic and rare. Idiopathic thrombocytopenic purpura in children usually a self limiting disorder. The diagnosis is usually made when there is thrombocytopenia and.
Listing a study does not mean it has been evaluated by the u. Primary immune thrombocytopenic purpura autoimmune thrombocytopenic purpura it is defined as an isolated thrombocytopenia with normal bone marrow in the absence of other causes of thrombocytopenia. Immune thrombocytopenia itp is a bleeding disorder caused by thrombocytopenia not associated with a systemic disease. Itp is a diagnosis of exclusion, characterized by isolated thrombocytopenia without a clinically apparent condition responsible for the low platelet count. Jan 11, 2020 immune thrombocytopenic purpura itp also known as idiopathic thrombocytopenic purpura and, more recently, as immune thrombocytopeniais a clinical syndrome in which a decreased number of circulating platelets thrombocytopenia manifests as a bleeding tendency, easy bruising purpura, or extravasation of blood from capillaries into skin an. The pathogenesis of immune thrombocytopaenic purpura. Immune thrombocytopenia itp is a fairly common blood disorder. Idiopathic thrombocytopenic purpura itp, also known as primary immune thrombocytopenia, is an autoimmune disorder characterized by isolated thrombocytopenia peripheral. They are defined by microangiopathic haemolytic anaemia and thrombocytopenia. Idiopathic thrombocytopenic purpura archives of disease. Evaluation of patients with suspected immune thrombocytopenic purpura. Intravenous antid treatment for immune thrombocytopenic. A platelet is a type of blood cell that helps the blood clot. The platelet disorder support association does not provide medical advice or endorse any medication, vitamins or herbs.
Symptoms may include bruising, nosebleed or bleeding in the mouth, bleeding into the skin, and abnormally heavy menstruation. Adults with immune thrombocytopenia purpura usually need treatment which is mainly in the form of medication. Corticosteroids are firstline treatment for immune thrombocytopenic purpura. Immunomodulatory treatments for persistent and chronic. Idiopathic thrombocytopenic purpura and mmr vaccine. Mmr vaccine and idiopathic thrombocytopenic purpura. These medicines, called steroids for short, help increase. Immune thrombocytopenic purpura itp stanford health care. Itp is a diagnosis of exclusion after other identifiable etiologies have been ruled out. Rapid strides have been made in the field of hematology, and advances in immune thrombocytopenic purpura itp management are no exception. Abnormalities in frequency and function of different immune cells can play a crucial role in this disease. Common variable immunodeficiency unmasked by treatment of.
Women are two to three times more likely than men to develop chronic itp. It may follow a viral infection or immunisation and is caused by an inappropriate response of the immune system. Intravenous antid treatment for immune thrombocytopenic purpura. Immune thrombocytopenia genetics home reference nih. Idiopathic thrombocytopenic purpura itp is a bleeding disorder characterized by too few platelets in the blood. Ppt idiopathic thrombocytopenic purpura itp powerpoint. Jan 25, 2015 idiopathic autoimmune thrombocytopenic purpuraitp the most common cause of acute onset of thrombocytopenia in an otherwise well child estimated about 1 in 20,000 children a recent history of viral illness is described in 5065% of cases of childhood itp 1252015 idiopathic thrombocytopenic purpura prof. The aim of this study was to evaluate the prognostic value of cd markers expressions by immune. Understanding immune thrombocytopenia itp immune thrombocytopenia thrombosightoepeaneeah, or itp, is a rare and potentially serious blood disorder. Ivig is an effective treatment for immune thrombocytopenic purpura itp that induces transient. Immune thrombocytopenia itp, formerly called idiopathic thrombocytopenic purpura, is a common acquired bleeding disordered characterized by isolated thrombocytopenia in the absence of a clinically. The estimated incidence is 100 cases per 1 million persons per year, and. These cells clump together at the site of a blood vessel injury in order to prevent blood loss.
This casecontrol study investigated immune thrombocytopenic purpura itp risk following live, inactivated. May, 2016 immune thrombocytopenia purpura associated with multiple myeloma. Idiopathic thrombocytopenia purpura is a blood disorder affecting the platelets. Immune thrombocytopenic purpura itp is a disorder in which antiplatelet antibodies cause accelerated destruction of platelets, resulting in thrombocytopenia and a varying propensity for bleeding. About 2030% of children will fail to remit over six months chronic idiopathic thrombocytopenic purpura. Symptoms can be mild, but for most patients the risk of. Adults tend to have the chronic longlasting type of itp. The goal of treatment in chronic itp is not well defined and depends on balancing. Immune thrombocytopenic purpura itp of childhood is an acquired immune mediated, and usually selflimited, condition of low platelet counts. Medicines often are used as the first course of treatment for both children and adults. Idiopathic thrombocytopenic purpura american academy of. The symptoms may follow a viral illness, such as chickenpox.
Immune thrombocytopenic purpura is an autoimmune disorder characterized by a low platelet count and mucocutaneous bleeding. Treatment may include a number of approaches, such as medications to boost your. Immune idiopathic thrombocytopenia purpura american. People with the disease have too few platelets in the blood. Management of adult idiopathic thrombocytopenic purpura. Thrombotic thrombocytopenic purpura associated with statin. For primary idiopathic itp, corticosteroids have been the standard firstline of treatment. Easy bruising of skin, mouth, gums petechiae red dots on the skin from broken capillaries nose bleeding, bleeding gums. People with mild thrombocytopenia might not need treatment. Immune thrombocytopenic purpura itp is an autoimmune condition that affects nearly 1. Immune thrombocytopenic purpura itp is a bleeding disorder characterized by isolated thrombocytopenia platelet count immune thrombocytopenia itp is an acquired thrombocytopenia, defined as a platelet count immune destruction of platelets. Primary immune thrombocytopenic purpura itp is an autoimmune disorder characterized by thrombocytopenia platelet count pc thrombocytopenia.
Immune thrombocytopenia or itp is a blood disorder in which the number of platelets in the blood is decreased to low levels. Platelets stick together clot to seal small cuts or breaks on blood vessel walls and stop bleeding. The adobe flash plugin is needed to view this content. Enter your email address to receive your free pdf download. Immune thrombocytopenic purpura itp is a relatively common autoimmune disorder in which antibodies are produced to circulating platelets. Immune thrombocytopenic purpura itp is an acquired bleeding disorder that is caused by abnormally low levels of platelets, which are necessary for normal blood clotting.
Corticosteroids corticosteerroids, such as prednisone, are commonly used to treat itp. For people who do need treatment for thrombocytopenia, treatment depends on its cause and how severe it is. American academy of pediatrics textbook of pediatric care, 2nd edition. Immune thrombocytopenic purpura, abbreviated itp, is a rare condition it is also known as idiopathic thrombocytopenic purpura. Idiopathic thrombocytopenic purpura itp marrow 50x. It can follow a virus, vaccination or certain medications, but for. Immune thrombocytopenia nord national organization for. When immune thrombocytopenia is a feature of other disorders, the condition is known as secondary immune thrombocytopenia. Immune thrombocytopenic purpura itp is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. Platelets are blood cells that allow the blood to clot properly. Itp is caused by antibodies mostly immunoglobulin g igg.
The management of thrombocytopenia in lupus patients is difficult, and corticosteroids and splenectomy are less effective than in primary immune thrombocytopenia 22. People with mild immune thrombocytopenia may need nothing more than regular monitoring and platelet checks. This can be unsafe because people depend on platelets to help stop bleeding. Thrombocytopenia american academy of family physicians. Update on the management of immune thrombocytopenic. It is traditionally defined by a platelet count of less than 100 x 109l, but treatment typically depends on symptomology rather than on the platelet count itself.
1156 812 303 940 655 262 1224 1259 993 993 725 512 39 1008 151 496 661 1503 320 776 761 466 1426 1402 1373 1395 303 105