Misdiagnosis of Chronic Thrombocytopenia in Childhood

Bader-Meunier, Brigitte; Proulle, Valérie; Trichet, Catherine; Debray, Dominique; Gabolde, Martine; Yvart, J; Dreyfus, Marie

doi:10.1097/00043426-200307000-00010

Cited by 43 publications

(31 citation statements)

References 18 publications

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“…In particular, it is necessary to reconsider a genetic thrombocytopenia (8.8 – A) [13,14,15,16,17,18,19,20] (table 4) and, periodically, all the forms related to other diseases not clearly evident previously, e.g. myelodysplastic and autoimmune syndromes or immunodeficiency (8.9 – A) [21,22,23,24,25,26,27,28].…”

Section: Resultsmentioning

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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Section: Resultsmentioning

confidence: 99%

“…The reexamination of peripheral blood smears is indicated for an in-depth evaluation of platelets, and red and white blood cells (8.5 – A) [13,14,15,16,17,18,19,20] (table 5). …”

Section: Resultsmentioning

confidence: 99%

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

et al. 2009

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“…168,169 Inherited disorders should be suspected if thrombocytopenia has been present since early life, a positive family history for a similar disorder is elicited, 170 or characteristic features are present.…”

Section: Differential Diagnosismentioning

confidence: 99%

International consensus report on the investigation and management of primary immune thrombocytopenia

Provan¹,

Stasi

Newland³

et al. 2010

Blood

1,832

1,954

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IntroductionPrimary immune thrombocytopenia (ITP) is an acquired immunemediated disorder characterized by isolated thrombocytopenia, defined as a peripheral blood platelet count less than 100 ϫ 10 9 /L, and the absence of any obvious initiating and/or underlying cause of the thrombocytopenia. 1 Until recently, the abbreviation ITP stood for idiopathic thrombocytopenic purpura, but current awareness relating to the immune-mediated nature of the disease, and the absence or minimal signs of bleeding in a large proportion of cases have led to a revision of the terminology. 1 Concepts surrounding the mechanisms of thrombocytopenia in ITP have shifted from the traditional view of increased platelet destruction mediated by autoantibodies to more complex mechanisms in which both impaired platelet production and T cell-mediated effects play a role. [2][3][4][5][6] Recent epidemiologic data suggest that the incidence in adults is approximately equal for the sexes except in the mid-adult years (30-60 years), when the disease is more prevalent in women. 7,8 ITP is classified by duration into newly diagnosed, persistent (3-12 months' duration) and chronic (Ն 12 months' duration). 1 Whereas ITP in adults typically has an insidious onset with no preceding viral or other illness and it normally follows a chronic course, 9 ITP in children is usually short-lived with at least two-thirds recovering spontaneously within 6 months. 10 Signs and symptoms vary widely. Many patients have either no symptoms or minimal bruising, whereas others experience serious bleeding, which may include gastrointestinal hemorrhage (GI), extensive skin and mucosal hemorrhage, or intracranial hemorrhage (ICH). The severity of thrombocytopenia correlates to some extent but not completely with the bleeding risk. 7,11 Additional factors (eg, age, lifestyle factors, uremia) affect the risk and should be evaluated before the appropriate management is determined.The investigation and management of ITP patients vary widely. The purpose of this consensus document is to comment on new data and provide recommendations relating to diagnosis and treatment. Final judgment regarding care of individual patients should, however, lie with the responsible health care professional and be based on careful investigation of individual circumstances. MethodsComposition of the panel. The panel included 22 members with recognized clinical and research expertise in ITP representing North America (United States, 7; Canada, 1), Europe (France, 1; Italy, 2; Spain, 1; Switzerland, 1; United Kingdom, 8), and Australia (1).Assessment of the literature. Articles were identified by a computer-assisted search of the literature published in English using the National Library of Medicine PubMed database. The For personal use only. on May 7, 2018. by guest www.bloodjournal.org From search criteria were: 'immune thrombocytopenic purpura', 'idiopathic thrombocytopenic purpura', 'ITP', and 'autoimmune thrombocytopenic purpura'. A subsequent search was performed using the corresponding MedLin...

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“…[10][11][12] VWD2B patients who lack the HMW multimers may also have moderate to severe thrombocytopenia, 13,14 sometimes associated with giant platelets and spontaneous platelet aggregates, [15][16][17] and this can predispose to more severe bleeding. Thrombocytopenia in VWD2B can be overlooked, especially in children [18][19][20] : it can be transient or persistent with variable platelet count on different occasions but usually worsens after conditions characterized by a long-or short-term rise in plasma VWF such as pregnancy, 21,22 infections, surgeries, 23 or DDAVP administration. 24 The thrombocytopenia phenotype is mainly caused by the enhanced 2BVWF-GpIb-␣ interactions resulting from gain of function of the A1 domain, leading to spontaneous platelet aggregation in vitro and in vivo.…”

Section: Introductionmentioning

confidence: 99%

Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients

Federici

Mannucci

Castaman

et al. 2009

Blood

234

308

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Type 2B von Willebrand disease (VWD2B)is caused by an abnormal von Willebrand factor (VWF) with increased affinity for the platelet receptor glycoprotein Ib-␣ (GPIb-␣) that may result in moderate to severe thrombocytopenia. We evaluated the prevalence and clinical and molecular predictors of thrombocytopenia in a cohort of 67 VWD2B patients from 38 unrelated families characterized by VWF mutations. Platelet count, mean platelet volume, and morphologic evaluations of blood smear were obtained at baseline and during physiologic (pregnancy) or pathologic (infections, surgeries) stress conditions. Thrombocytopenia was found in 20 patients (30%) at baseline and in 38 (57%) after stress conditions, whereas platelet counts were always normal in 16 patients (24%) from 5 families carrying the P1266L/Q or R1308L mutations. VWF in its GPIb-␣-binding conformation (VWFGPIb-␣/BC) was higher than normal in all except the 16 cases without thrombocytopenia (values up to 6-fold higher than controls). The risk of bleeding was higher in patients with thrombocytopenia (adjusted hazard ratio ‫؍‬ 4.57; 95% confidence interval, 1.17-17.90) and in those with the highest tertile of bleeding severity score (5.66; 95% confidence interval, 1.03-31.07). Prediction of possible thrombocytopenia in VWD2B by measuring VWF-GPIb-␣/BC is important because a low platelet count is an independent risk factor for bleeding. (Blood. 2009;113: 526-534)

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Misdiagnosis of Chronic Thrombocytopenia in Childhood

Cited by 43 publications

References 18 publications

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines

International consensus report on the investigation and management of primary immune thrombocytopenia

Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients

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