Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children

S, Aronis; H, Platokouki; Avgeri, M; H, Pergantou; Keramidas, D

doi:10.1080/08035250310024718

Cited by 10 publications

(13 citation statements)

References 17 publications

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Section: Discussionsupporting

confidence: 82%

“…The reported response rates with regard to achieving a sustained increase in platelet count varied from 40 to 85% [7,22,23,24,25,26,27]. In this study, the sustained response (CR + PR) rate was 76.9%, which is in agreement with the previous reports [7,22,23,24,25,26,27]. Among the responders, 13.8% of the patients in our study subsequently relapsed, which is equal to or somewhat lower than what is reported in other studies [23, 27].…”

Section: Discussionmentioning

confidence: 99%

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Splenectomy for Chronic Idiopathic Thrombocytopenic Purpura in Children: A Single Center Study in China

Wang¹,

et al. 2006

Acta Haematol

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The management of chronic and refractory idiopathic thrombocytopenic purpura (ITP) in children is controversial. We conducted a retrospective review of our single center experience in China between 1990 and 2003 with splenectomy for chronic ITP in children in order to determine the initial and long-term hematological response, morbidity, mortality, predictors of response to splenectomy and the therapy in children who failed splenectomy. Of 65 children analyzed, the overall immediate clinical response to splenectomy was 89.2%. The median postsplenectomy follow-up time was 52 months (8–124). During follow-up, 9 children (13.8%) relapsed within a median time of 6 months (2–58). The overall morbidity was 1.5% and perioperative mortality was zero. During follow-up, 1 child died of intracranial hemorrhage (ICH) and 1 died of overwhelming postsplenectomy infection (OPSI). The platelet count at day 7 after splenectomy was a predictor of a sustained response to splenectomy but no preoperative parameters were predictors of the response to splenectomy. Of the 15 children who failed splenectomy, excluding the one who died of ICH, only 2 children intermittently required corticosteroids and IVIG. Splenectomy is a potential therapy to provide long-term control of disease in children with chronic ITP and is associated with low morbidity and mortality. The risk of fulminant sepsis remains an omnipresent concern. Antipneumococcal vaccination and antibiotic prophylaxis should be recommended and children should receive timely and adequate antibiotics for bacteria infection to lessen the problem of OPSI.

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Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Splenectomy for Chronic Idiopathic Thrombocytopenic Purpura in Children: A Single Center Study in China

Wang¹,

et al. 2006

Acta Haematol

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“…American guidelines state that splenectomy should be performed in patients with chronic symptomatic thrombocytopenia lasting >12 months and a platelet count <10 × 10 9 /l being unresponsive to standard treatment and presenting no surgical contraindications [77]. The reported effectiveness varies from 70 to 89.2% in a limited number of retrospective studies, with different definitions of treatment response and variable follow-up [10,140,141,142,143,144,145,146,147,148]. Some authors state that a positive treatment response to IVIGs predicts the positive response to a splenectomy [143,149,150,151], but this observation has not been confirmed by others [152,153,154,155,156].…”

Section: Treatmentsmentioning

confidence: 99%

“…There are specific recommendations about immunization of children before undergoing splenectomy [76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111,112,113,114,115,116,117,118,119,120,121,122,123,124,125,126,127,128,129,130,131,132,133,134,135,136,137,138,139,140,141,142,143,144,145,146,147,148,149,150,151,152,153,154,155,156,157,158,159,160,161,162,163,164,165,166,167<...>…”

Section: Treatmentsmentioning

confidence: 99%

“…Treatment aiming at definitive remission (8.0 – B) consists of splenectomy [10,139,140,141,142,143,144,145,146,147,148,149,150,151,152,153,154,155,156,157,158]. Splenectomy should be performed in children with chronic ITP [10,140,141,142]> 5–6 years of age, preferably with a disease lasting at least 4 years, being symptomatic or with low quality of life, following failure of medical treatment, due to dependency or excess toxicity (8.9 – A).…”

Section: Treatmentsmentioning

confidence: 99%

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Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

et al. 2009

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Background/Objective: The management of chronic childhood idiopathic thrombocytopenic purpura (ITP) is distinct from acute ITP. Similar to the publication on acute ITP guidelines, the AIEOP (Associazione Italiana di Ematologia e Oncologia Pediatrica) considered it appropriate to develop consensus guidelines for chronic childhood ITP to provide useful and shared information for physicians, healthcare professionals, parents and patients. Design/Methods: A preliminary, evidence-based document issued by a select group of AIEOP pediatric hematologists was discussed, modified and approved during a Consensus Conference according to procedures previously validated by the AIEOP Board. Results: The guidelines give prominence to the periodical reevaluation of all the etiological hypotheses of thrombocytopenia in relation to its clinical condition. The majority of chronic ITP children do not require treatment, especially if bleeding is absent or minimal. The treatment decision depends on several factors other than the platelet count, and treatment options are suggested in relation to the therapeutic scenarios. Recommendations are given regarding support for surgery, particular hemorrhagic conditions, daily activities/sports, as well as for vaccines and drugs. Experimental treatments are also discussed.

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Diagnosis and Management of Autoimmune Cytopenias in Childhood

Teachey

Lambert

2013

Pediatric Clinics of North America

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The diagnosis and management of children with autoimmune cytopenias can be challenging, as this diagnostic category encompasses a number of heterogeneous but closely related conditions. Children can present with immune-mediated destruction of a single cell lineage or multiple cell lineages, including platelets (immune thrombocytopenia, ITP), erythrocytes (autoimmune hemolytic anemia), and neutrophils (autoimmune neutropenia). Immune-mediated destruction can be primary or secondary to a co-morbid immunodeficiency, malignancy, rheumatologic condition, or lymphoproliferative disorder. Despite the heterogeneity, treatment options are similar and generally consist of non-specific immune suppression or modulation. Fortunately, this non-specific approach is changing as recent insights into disease biology have led to targeted therapies for certain patients, including the use of thrombopoietin mimetics in ITP and sirolimus for cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS).

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Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children

Cited by 10 publications

References 17 publications

Splenectomy for Chronic Idiopathic Thrombocytopenic Purpura in Children: A Single Center Study in China

Splenectomy for Chronic Idiopathic Thrombocytopenic Purpura in Children: A Single Center Study in China

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

Diagnosis and Management of Autoimmune Cytopenias in Childhood

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