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Idiopathic Thrombocytopenic Purpura (Thrombocytopenic Purpura, Autoimmune)

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Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.

Also Known As:

Thrombocytopenic Purpura, Autoimmune; Purpura, Thrombocytopenic, Idiopathic; Autoimmune Thrombocytopenia; Werlhof Disease; Autoimmune Thrombocytopenias; Autoimmune Thrombocytopenic Purpuras; Disease, Werlhof; Disease, Werlhof's; Idiopathic Thrombocytopenic Purpuras; Purpura, Autoimmune Thrombocytopenic; Purpura, Idiopathic Thrombocytopenic; Purpuras, Autoimmune Thrombocytopenic; Purpuras, Idiopathic Thrombocytopenic; Thrombocytopenia, Autoimmune; Thrombocytopenias, Autoimmune; Thrombocytopenic Purpura, Idiopathic; Thrombocytopenic Purpuras, Autoimmune; Thrombocytopenic Purpuras, Idiopathic; Werlhofs Disease; Autoimmune Thrombocytopenic Purpura; Purpura, Thrombocytopenic, Autoimmune; Werlhof's Disease

Networked: 3202 relevant articles (178 outcomes, 236 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombocytopenia (Thrombopenia)
2.	Autoimmune Hemolytic Anemia (Cold Agglutinin Disease)
3.	Mucocutaneous Lymph Node Syndrome (Kawasaki Disease)
4.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
5.	Hemolytic Anemia

Experts

1.	Hou, Ming: 29 articles (08/2011 - 08/2002)
2.	Peng, Jun: 17 articles (11/2009 - 06/2003)
3.	Yang, Renchi: 16 articles (01/2011 - 05/2004)
4.	Stasi, Roberto: 14 articles (05/2011 - 05/2002)
5.	Ikeda, Yasuo: 13 articles (10/2008 - 05/2002)
6.	Kuwana, Masataka: 11 articles (04/2012 - 08/2002)
7.	Wang, Lin: 11 articles (09/2008 - 04/2005)
8.	Salama, Abdulgabar: 10 articles (09/2015 - 07/2004)
9.	Buchanan, George R: 10 articles (11/2011 - 08/2002)
10.	Franchini, Massimo: 10 articles (01/2010 - 11/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Idiopathic Thrombocytopenic Purpura:

1.	Inosine Triphosphate (ITP)IBA 05/01/2008 - "Several recent studies have suggested that an association exists between Helicobacter pylori (HP) eradication and improvement in platelet count in a significant proportion of patients with idiopathic thrombocytopenic purpura (ITP). " 02/01/1981 - "A significant reduction in the proportion and absolute number of circulating T gamma (suppressor) lymphocytes was observed in 15 adult patients with idiopathic thrombocytopenic purpura (ITP). " 04/01/2001 - "When a patient is steroid-dependant, a currently available strategy in chronic idiopathic thrombocytopenic purpura (ITP) is to follow a trial and error approach with any of the known drugs which has been found effective in the condition. " 08/01/1996 - "Clinical studies employing patients presenting with idiopathic thrombocytopenic purpura (ITP) indicate that this new form of therapy is effective in approximately 40% of treated patients. " 08/01/2009 - "Helicobacter pylori eradication is useful for improvement of a half of patients with idiopathic thrombocytopenic purpura (ITP), but its long-term therapeutic efficacy has not been elucidated. "
2.	Intravenous Immunoglobulins (IVIG)FDA Link 06/01/2004 - "It was suggested that DFPP combined with corticosteroid and IVIg was effective for severe autoimmune thrombocytopenia on SLE patients refractory to the conventional therapies." 05/01/2000 - "IVIg was first demonstrated to be effective in auto-immune desorders twenty years ago in the treatment of idiopathic thrombocytopenic purpura. " 08/01/1997 - "It has been over 15 years since IVIG was first shown to be effective in the treatment of patients with autoimmune thrombocytopenic purpura. " 01/01/2002 - "Many studies confirmed the efficacy of IVIG in the treatment of idiopathic thrombocytopenic purpura and other autoimmune disorders. " 06/01/2003 - "Since the first study of Paul Imbach who demonstrated the beneficial effect in idiopathic thrombocytopenic purpura, IVIg is also used in a number of autoimmune and inflammatory diseases. "
3.	Immunoglobulins (Immunoglobulin)IBA 02/01/1995 - "Failure of repeated courses of high-dose intravenous immunoglobulin to induce stable remission in patients with chronic idiopathic thrombocytopenic purpura." 07/01/1987 - "Transient efficacy of high-dose intravenous immunoglobulin infusions (HDI) is widely demonstrated in autoimmune thrombocytopenic purpura (AITP). " 12/01/2010 - "The efficacy of different dose intravenous immunoglobulin in treating acute idiopathic thrombocytopenic purpura: a meta-analysis of 13 randomized controlled trials." 01/01/1997 - "Assessment of the efficacy of a last-generation polyvalent immunoglobulin in the treatment of idiopathic thrombocytopenic purpura." 12/01/2010 - "The purpose of this study was to compare the effects of different dose intravenous immunoglobulin for treatment of acute idiopathic thrombocytopenic purpura. "
4.	rituximab (Mabthera)FDA Link 02/01/2010 - "Rituximab was also effective in patients with autoimmune thrombocytopenia, inducing a significant increase in platelet counts (p = 0.012). " 08/01/2012 - "[Clinical therapeutic efficacy of rituximab for relapsed and refractory idiopathic thrombocytopenic purpura]." 10/01/2014 - "A multi-centre, single-arm, open-label study evaluating the safety and efficacy of fixed dose rituximab in patients with refractory, relapsed or chronic idiopathic thrombocytopenic purpura (R-ITP1000 study)." 07/01/2015 - "Efficacy of standard dose rituximab for refractory idiopathic thrombocytopenic purpura in children." 03/01/2010 - "[Efficacy of lower dose rituximab therapy for idiopathic thrombocytopenic purpura.]."
5.	Danazol (Azol)FDA LinkGeneric 08/01/1987 - "Although danazol is effective in the treatment of idiopathic thrombocytopenic purpura, its long-term safety and optimal dosage are not well established. " 03/01/1987 - "Thus, danazol appears to be an effective immune modulator, correcting the abnormality of T-cell subsets seen in autoimmune thrombocytopenia by increasing the percentage of Thi lymphocytes." 06/01/1992 - "Danazol therapy in idiopathic thrombocytopenic purpura: the efficacy of low-medium dose therapy." 11/01/1989 - "Long-term danazol therapy in autoimmune thrombocytopenia: unmaintained remission and age-dependent response in women." 10/01/2008 - "[Danazol - effective second line therapy in idiopathic thrombocytopenic purpura in children. "
6.	Immunoglobulin G (IgG)IBA 01/01/1979 - "Studied were 364 blood samples drawn from 196 patients; 173 patients had idiopathic thrombocytopenic purpura; 65 (93 %) of 70 in the acute phase of their disease had elevated PBIgG (values up to 100 times greater than the normal); 98 (76.5 %) of 128 blood samples from patients during a chronic phase had abnormally high PBIgG but the levels of IgG were generally lower than those observed in the acute phase; 75 (75 %) of 100 samples from patients in remission did not have pathologically increased PBIgG. " 11/01/1986 - "Treatment of acute idiopathic thrombocytopenic purpura of childhood with intravenous immunoglobulin G: comparative efficacy of 7S and 5S preparations." 11/01/1986 - "A prospective randomized study comparing 7S immunoglobulin G to a 5S IgG preparation for therapy of acute idiopathic thrombocytopenic purpura was conducted. " 01/15/1983 - "A study of the effect of high-dose intravenous IgG in 25 adults with autoimmune thrombocytopenia confirmed the predictable rise in the platelet count seen during the infusion and previously reported in children. " 03/18/2008 - "We demonstrate here that EndoS indeed has a protective effect in a mouse model of lethal IgG-driven immune (or idiopathic) thrombocytopenic purpura. "
7.	Dexamethasone (Maxidex)FDA LinkGeneric 09/01/1997 - "Recently, pulsed high-dose dexamethasone (HDD) has been shown to be effective in the treatment of autoimmune thrombocytopenic purpura (AITP). " 01/01/1997 - "To determine the efficacy of high-dose dexamethasone in chronic idiopathic thrombocytopenic purpura of childhood. " 02/01/1996 - "Because high-dose oral dexamethasone therapy has been reported to be effective for adults with idiopathic thrombocytopenic purpura, we assessed the short-term efficacy and toxicity of dexamethasone in seven children with chronic or refractory idiopathic thrombocytopenic purpura. " 06/02/1994 - "Although the possibility of spontaneous remission and a delayed benefit from prior therapy cannot be excluded in this small group of patients, pulsed high-dose treatment with dexamethasone may provide a low-cost therapeutic option with minimal side effects in patients with refractory idiopathic thrombocytopenic purpura." 01/01/1997 - "A trial of high-dose dexamethasone therapy for chronic idiopathic thrombocytopenic purpura in childhood."
8.	eltrombopag FDA Link 03/01/2014 - "Eltrombopag was suggested to be effective in perioperative management in a patient with idiopathic thrombocytopenic purpura undergoing cardiovascular surgery. " 09/01/2012 - "Prolonged remission from eltrombopag in chronic refractory idiopathic thrombocytopenic purpura." 03/01/2014 - "[Effectiveness of perioperative administration of eltrombopag in a patient with idiopathic thrombocytopenic purpura undergoing cardiovascular surgery]." 01/01/2013 - "The authors' reply to Allen et al.: "A Comment on Boyers et al.: 'eltrombopag for the treatment of chronic immune or idiopathic thrombocytopenic purpura: a NICE single technology appraisal'"." 01/01/2013 - "A comment on Boyers et al.: "eltrombopag for the treatment of chronic immune or idiopathic thrombocytopenic purpura: a NICE single technology appraisal"."
9.	Prednisone (Sone)FDA LinkGeneric 02/01/2004 - "In conclusion, combination of multiglycosidorum triptergii with prednisone is effective and safe for treatment of chronic refractory idiopathic thrombocytopenic purpura in children." 12/01/2008 - "Platelet production rate predicts the response to prednisone therapy in patients with idiopathic thrombocytopenic purpura." 07/01/2008 - "Multidrug resistance-1 in T lymphocytes and natural killer cells of adults with idiopathic thrombocytopenic purpura: effect of prednisone treatment." 01/01/2008 - "We list the case report of a young patient with congenital FVII deficiency (FVII level < 1%) and acute idiopathic thrombocytopenic purpura with yet unknown aetiology of which has not been fully clarified although the very favourable effect of prednisone therapy indicates the autoimmune mechanism." 09/01/1996 - "The chronic autoimmune thrombocytopenia and recurrent phases of autoimmune haemolytic anaemia did, however, respond to high-dose prednisone. "
10.	Prednisolone (Predate)FDA LinkGeneric 06/01/2008 - "A 64-year-old Japanese woman suffering from idiopathic thrombocytopenic purpura was treated with prednisolone. " 11/01/2001 - "A diagnosis of idiopathic thrombocytopenic purpura was made, and oral prednisolone (1 mg/kg/day) was administered immediately. " 01/01/2000 - "[Low-dose prednisolone therapy for idiopathic thrombocytopenic purpura]." 07/01/1996 - "She was diagnosed as idiopathic thrombocytopenic purpura and a standard dose of prednisolone was started immediately. " 02/01/1993 - "[Long-term maintenance of platelets by IFN alpha-2b in a case of chronic idiopathic thrombocytopenic purpura refractory to prednisolone therapy]."

Therapies and Procedures

1.	Splenectomy 08/01/2006 - "Splenectomy is the most effective treatment for patients with severe autoimmune thrombocytopenia (AITP) who do not have a spontaneous or drug- induced remission. " 10/01/1985 - "It is concluded that splenectomy is beneficial in most adult patients with idiopathic thrombocytopenic purpura and that radiological techniques to measure the size of the spleen may be useful in predicting which patients may relapse." 12/01/1996 - "A prospective clinical study was undertaken to evaluate the rate of complete remission in idiopathic thrombocytopenic purpura after splenectomy, to reveal the influence of preoperative immunosuppression on the postoperative course in groups of patients with different responses to treatment, and to describe possible prognostic factors predicting the postoperative course of idiopathic thrombocytopenic purpura. " 01/01/1988 - "In the other patient who had idiopathic thrombocytopenic purpura, a successful accessory splenectomy was done at the same time as her splenectomy, and was followed by 6 months' complete remission. " 10/15/2006 - "Splenectomy and spontaneous remission in children with chronic idiopathic thrombocytopenic purpura."
2.	Drug Therapy (Chemotherapy) 09/20/2007 - "Long-term outcomes of combined chemotherapy in chronic refractory idiopathic thrombocytopenic purpura." 01/01/2003 - "Chemotherapy in a patient with prior history of idiopathic thrombocytopenic purpura." 05/18/1978 - "Selective chemotherapy of macrophages in the treatment of idiopathic thrombocytopenic purpura." 10/01/2000 - "Autoimmune thrombocytopenia after high-dose chemotherapy and autologous bone marrow/peripheral blood stem cell transplantation occurs infrequently and only six cases meeting the criteria have been reported in the literature. " 01/01/1983 - "We present data on 5 cases with autoimmune thrombocytopenic purpura treated with plasma exchange in whom conventional drug therapy had been ineffective. "
3.	Transplants (Transplant) 09/18/1982 - "This study suggests that the immune defect in autoimmune thrombocytopenia can be transferred in cells capable of proliferating in a bone-marrow graft." 08/01/1997 - "Marrow graft was rejected early; however, a second attempt using bone marrow from the mother led to engraftment and to control of lymphoproliferation and of autoimmune thrombocytopenia up to the last follow-up at 24 months after BMT. " 09/18/1982 - "Autoimmune thrombocytopenia acquired from an allogeneic bone-marrow graft." 11/01/1996 - "[Case report of coronary artery bypass grafting using only arterial grafts in a patient with idiopathic thrombocytopenic purpura]." 12/01/2011 - "Autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura in pediatric solid organ transplant recipients, report of five cases and review of the literature."
4.	Platelet Transfusion (Blood Platelet Transfusions) 01/01/1994 - "Despite anecdotal reports of utility in several hematologic syndromes, publications of clinical trials are available only for autoimmune thrombocytopenic purpura (AITP) and refractoriness to platelet transfusions (RFT) associated with alloimmunization. " 03/01/1988 - "One patient received platelet transfusions for counts less than 50 x 109)/L, one patient developed a clinical picture of idiopathic thrombocytopenic purpura, and six patients maintained a platelet count greater than 20 x 10(9)/L at a median of 23 days (range 14 to 25 days) following stem cell infusion. " 10/01/1987 - "[A case of idiopathic thrombocytopenic purpura responding to high-dose globulin therapy following platelet transfusion with globulin therapy]." 06/01/1986 - "Urgent treatment of idiopathic thrombocytopenic purpura with single-dose gammaglobulin infusion followed by platelet transfusion." 11/22/1952 - "Platelet transfusions and the pathogenesis of idiopathic thrombocytopenic purpura."
5.	Aftercare (After-Treatment) 07/01/1987 - "Persistent remission of adult chronic autoimmune thrombocytopenic purpura after treatment with high-dose intravenous immunoglobulin." 08/01/2008 - "This study was aimed to investigate the expression level of CD4(+) CD25(high) T cells in peripheral blood of patients with idiopathic thrombocytopenic purpura (ITP) before and after treatment and to explore its significance in pathogenesis of ITP. " 01/01/2015 - "We aimed to explore the changes of peripheral B1 cells before and after treatment of adult idiopathic thrombocytopenic purpura (ITP) and to investigate the association of these changes with the disease condition and prognosis. " 02/01/2010 - "Idiopathic thrombocytopenic purpura after treatment failure: romiplostim is a better option." 04/01/2003 - "To clarify the prognostic contribution of peripheral blood cell and bone marrow megakaryocyte counts in patients with idiopathic thrombocytopenic purpura, a series of data of 299 ITP patients including the counts of peripheral white blood cells and platelets, their increase potentials after treatment and the megakaryocytic counts on the bone marrow smears at diagnosis as well were collected and retrospectively analyzed to correlate with the disease development. "