Management of patients with chronic, refractory idiopathic thrombocytopenic purpura

George, James N.; Kojouri, Kiarash; Perdue, Jedidiah J.; Vesely, Sara K.

doi:10.1053/shem.2000.8955

Cited by 21 publications

(30 citation statements)

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“…Patients affected by IPT in the absence of severe hemorrhage are monitored and don't need specific medicinal therapy 3,[17][18][19] , a fact which was verified with this patient.…”

Section: Discussionsupporting

confidence: 61%

“…In these cases follow-up without medication intervention is the best form of treatment and success of intermittent controls of platelet count until the disease remission 3,[17][18][19] . In more severe cases with risks of intracranial, gastro-intestinal and genitourinary hemorrhage, there is the option of platelet transfusion 3,11,[18][19][20] . In cases of chronic IPT, in which no success was achieved with all the attempted therapeutic resources, splenectomy 3 is recommended, despite its performance restricted success 20 .…”

Section: Introductionmentioning

confidence: 99%

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Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

Rossier

Vieira

Ciamponi

et al. 2015

RGO, Rev. Gaúch. Odontol.

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RESUMODentre as hemopatias a púrpura trombocitopênica idiopática (PTI) se enquadra no grupo de doenças com alterações plaquetárias e se caracteriza pela diminuição no número de plaquetas do sangue. Clinicamente, crianças com PTI podem apresentar petéquias, equimose, hematúria, epistaxe e ocasionalmente hemorragias teciduais. As manifestações bucais, quando presentes, se caracterizam pelo sangramento gengival espontâneo e petéquias ou hematomas na mucosa, palato e língua. Portanto, é fundamental que o cirurgião-dentista tenha conhecimento sobre a patologia e terapêutica necessária e assim possa determinar o tratamento ideal para estes pacientes. O objetivo do trabalho é relatar o caso de um paciente de 4 anos de idade com PTI aguda, discutir as principais manifestações clínicas em crianças e orientar o cirurgião-dentista para o correto manejo destes pacientes.

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“…Patients affected by IPT in the absence of severe hemorrhage are monitored and don't need specific medicinal therapy 3,[17][18][19] , a fact which was verified with this patient.…”

Section: Discussionsupporting

confidence: 61%

Section: Introductionmentioning

confidence: 99%

Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

Rossier

Vieira

Ciamponi

et al. 2015

RGO, Rev. Gaúch. Odontol.

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“…While conventional therapies such as corticosteroids and splenectomy primarily focus on reducing platelet destruction [30,31], the finding that platelet production is suboptimal in a substantial proportion of patients with ITP [32,33] has led to development of the TPO mimetics, which enhance platelet production and may therefore provide better outcomes [34].…”

Section: New Agents Have Changed the Treatment Paradigmmentioning

confidence: 99%

Should medical treatment options be exhausted before splenectomy is performed in adult ITP patients? A debate

et al. 2010

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Patients with primary immune thrombocytopenia (ITP) may require treatment to reduce the risk of serious bleeding if platelets remain consistently below 30 x 109/L. While approximately 70-80% of patients respond to an initial course of corticosteroids, relapse is common. For steroid-refractory patients, there is a choice between surgical splenectomy and further medical treatments, based on many factors including the patient's bleeding history, fitness for surgery, comorbidities, tolerance of adverse events, lifestyle and preferences. Treatments that have traditionally been used (corticosteroids, azathioprine, danazol) suppress the immune system, potentially predisposing patients to infection. Recent insights into the underlying pathophysiology of the disease have allowed the development of targeted therapies, including the thrombopoietin (TPO) receptor agonists, which enhance platelet production. Phase III trials have found romiplostim and eltrombopag to be well tolerated and effective in elevating platelet counts and reducing bleeding in both splenectomised and nonsplenectomised patients with chronic ITP. The B-cell targeted monoclonal antibody rituximab has also shown some potential in this setting, although data are currently limited and there are toxicity concerns. The decision whether to proceed to splenectomy or try other medical therapies in corticosteroidrefractory patients remains patient-specific. Splenectomy has its risks (including perioperative and long-term risks), and relapse/nonresponse are relatively common, but it offers the possibility of cure in the majority of patients. However, newer treatments may potentially allow splenectomy to be deferred for prolonged periods, as well as providing alternative treatment options for patients who fail splenectomy . 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 However, newer treatments may potentially allow splenectomy to be deferred for prolonged periods, as well as providing alternative treatment options for patients who fail splenectomy.

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“…The clinical syndromes of ITP are distinct between children and adults: Childhood ITP characteristically is acute in onset and resolves spontaneously in most cases within 6 months, 1 whereas adult ITP typically has an insidious onset and rarely resolves spontaneously. [2][3][4][5] The incidence of ITP in children is estimated to be approximately 46 new cases per million per year. Patients who fail to maintain a normal platelet count after conventional therapies are labeled as chronic refractory ITP patients.…”

mentioning

confidence: 99%

“…Treatment modalities include glucocorticoids, splenectomy, intravenous immunoglobulin (IVIG), anti-Rh (D) immune globulin, azathioprine, cyclophosphamide, vinca alkaloids, danazol, vitamin C, combination chemotherapy, cyclosporine, dapsone and interferon-␣-2b. [1][2][3][4][5] Recent information suggests that the anti-CD20 antibody, rituximab may be beneficial. 6 To date, limited information exists on the use of hematopoietic progenitor cell transplantation as a treatment for this disease.…”

mentioning

confidence: 99%

Resolution of chronic idiopathic thrombocytopenia purpura following syngeneic peripheral blood progenitor transplant

Zaydan

Turner

Miller

2002

Bone Marrow Transplant

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Summary:Idiopathic thrombocytopenia purpura (ITP) is an acquired disease of children and adults characterized by a low platelet count, an essentially normal bone marrow, and absence of evidence for other disease. We report the use of syngeneic peripheral blood progenitor transplantation (PBPT) in a 19-year-old male with chronic refractory ITP since the age of 5. Engraftment was successful and has resulted in resolution of his disease. We conclude that syngeneic PBPT is a potentially curative option for refractory ITP.

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Management of patients with chronic, refractory idiopathic thrombocytopenic purpura

Cited by 21 publications

References 0 publications

Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

Should medical treatment options be exhausted before splenectomy is performed in adult ITP patients? A debate

Resolution of chronic idiopathic thrombocytopenia purpura following syngeneic peripheral blood progenitor transplant

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