Materiały źródłowe

• #1 A clinical prediction score for transient versus persistent childhood immune thrombocytopenia – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33058474/

  Essentials There is a need for improved tools to predict persistent and chronic immune thrombocytopenia (ITP). […] The prognosis (transient, persistent, or chronic ITP) remains difficult to predict. […] The Childhood ITP Recovery Score predicts transient vs. persistent ITP and response to intravenous immunoglobulins. […] In external validation, the score predicted transient versus persistent ITP at 3 months follow-up with an area under the receiver operating characteristic curve of 0.71. […] The score also predicted cessation of bleeding symptoms and the response to intravenous immunoglobulins (IVIg). […] The Childhood ITP Recovery Score predicts prognosis and may be useful to individualize clinical management.

• #1 How we treat primary immune thrombocytopenia in adults | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01401-z

  ITP remains a diagnosis of exclusion due to the lack of a “gold standard” diagnostic test. […] According to several population-based studies, myelodysplastic syndrome (MDS) and secondary ITP, such as thrombocytopenias due to systemic autoimmune diseases, malignancies, primary immune deficiency, or drug exposure, accounted for approximately 20% of the misdiagnosis. […] According to the disease duration, ITP can be divided into newly diagnosed (<3 months), persistent (3–12 months), and chronic (>12 months) phases. Adult patients are more likely to develop chronic disease than children. […] Moreover, higher platelet counts at diagnosis and the presence of ANAs and anti-thyroid antibodies may serve as predictors of disease chronicity. […] Identification of patients at high risk of severe bleeding will help start the treatment timely to prevent fatal hemorrhage, even in those with higher platelet counts, whereas this remains a big challenge.

• #1 Immune Thrombocytopenia (ITP): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202158-overview

  Prognosis varies in children and adults. […] More than 80% of children with untreated immune thrombocytopenia (ITP) have a spontaneous recovery with completely normal platelet counts in 2-8 weeks. Fatal bleeding occurs in 0.9% upon initial presentation. […] A systematic review and meta-analysis identified the following factors associated with higher risk of ITP in children becoming chronic: Female sex (odds ratio [OR] 1.17), Age 11 years at presentation (OR 2.47), No preceding infection or vaccination (OR 3.08), Insidious onset (OR 11.27), Platelet count 20 109/L at presentation (OR 2.15), Presence of antinuclear antibodies (OR 2.87), Treatment with methylprednisolone plus intravenous immunoglobulin (OR 2.67). […] Factors associated with lower likelihood of developing chronic ITP were as follows: Mucosal bleeding at diagnosis (OR 0.39), Treatment with intravenous immunoglobulin alone (OR 0.71).

• #1 Immune Thrombocytopenia (ITP): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/202158-overview

  Approximately 60-90% of adults with ITP respond with an increased platelet count after treatment with prednisone or prednisone and IV RhIG or IVIG. Of those adults who do not maintain an increased platelet count and who require splenectomy, approximately two thirds have a sustained response and 10-15% have a partial response.

• #1 How we treat primary immune thrombocytopenia in adults | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01401-z

  Older age, low platelet counts, the presence of comorbidities, and concurrent anticoagulants or antiplatelet medications contribute to an increased risk of bleeding. […] Data from the CARMEN-France registry indicated that anticoagulant exposure was the major risk factor for severe bleeding in very elderly ITP patients aged ≥80 years, and thus for whom more close monitoring was needed. […] Disease course also impacts bleeding manifestations, and the rate of bleeding-related events in newly diagnosed patients is significantly higher than in chronic cases. […] A Chinese nationwide multicenter study reported that the mortality rate of ICH in adult ITP was 33.8%, and the risk factors of mortality included intraparenchymal hemorrhage, platelet counts ≤10 × 10^9/L, serious infections, severe preceding bleeding events, and Glasgow coma scale.

• #2 How we treat primary immune thrombocytopenia in adults | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01401-z

  ITP remains a diagnosis of exclusion due to the lack of a “gold standard” diagnostic test. […] According to several population-based studies, myelodysplastic syndrome (MDS) and secondary ITP, such as thrombocytopenias due to systemic autoimmune diseases, malignancies, primary immune deficiency, or drug exposure, accounted for approximately 20% of the misdiagnosis. […] According to the disease duration, ITP can be divided into newly diagnosed (<3 months), persistent (3–12 months), and chronic (>12 months) phases. Adult patients are more likely to develop chronic disease than children. […] Moreover, higher platelet counts at diagnosis and the presence of ANAs and anti-thyroid antibodies may serve as predictors of disease chronicity. […] Identification of patients at high risk of severe bleeding will help start the treatment timely to prevent fatal hemorrhage, even in those with higher platelet counts, whereas this remains a big challenge.

• #2

  https://haematologica.org/article/view/7816

  This prospective observational cohort study aimed to explore the clinical features of incident immune thrombocytopenia in adults and predictors of outcome, while determining if a family history of autoimmune disorder is a risk factor for immune thrombocytopenia. […] After 12 months, only 37% of patients not subject to disease-modifying interventions achieved cure. […] Immune thrombocytopenia in adults has been shown to progress to a chronic form in the majority of patients. […] A lower platelet count could be indicative of a more favorable outcome. […] This confirms previous data suggesting that ITP is more likely to follow a chronic course in affected adults, compared to children in whom cure is achieved within several weeks in most patients. […] Our results confirm that sex and age are not risk factors for chronicity.

• #2 How we treat primary immune thrombocytopenia in adults | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01401-z

  The main goals of ITP treatment are to prevent bleeding and maintain the platelet count above a safe level to minimize the bleeding tendency. […] The updated ASH guidelines recommend the initiation of disease-specific treatments if the platelet counts are <30 × 10^9/L, while it is a recommendation based on very low certainty in the evidence of effects. [...] The association between platelet count and bleeding events is poor in ITP patients with platelet counts >10 × 10^9/L. […] Although newly diagnosed ITP patients have relatively high response rates to initial treatments, the vast majority of them undergo disease relapse, and about 60–70% of the patients will progress to persistent or chronic ITP. […] The reported overall response rates of these above-mentioned thrombopoietic agents range from 70 to 90% in RCTs for previously treated chronic patients, and 50–60% of them could maintain the response with prolonged treatment duration.

• #3

  https://haematologica.org/article/view/7816

  Interestingly, a higher platelet count at baseline was found to be associated with increased odds of chronicity at 12 months, independent of confounders. […] However, it did not reach the 5% level of significance and further studies are needed to confirm such an association, which was also found when considering all patients recovering from ITP, regardless of disease-modifying treatment. […] The predictors of chronicity were explored prospectively for the first time in adults, suggesting that higher platelet counts at diagnosis are negatively correlated with the risk of chronicity. […] However, further research is needed to confirm this finding and potentially to identify other predictive factors for chronicity at the time of diagnosis.

• #3 How we treat primary immune thrombocytopenia in adults | Journal of Hematology & Oncology | Full Text

  https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01401-z

  A more recent meta-analysis including more RCTs also revealed that TPO-RAs had significantly lower treatment failure and all-cause mortality rates than the placebo, and TPO-RAs did not increase the occurrence of adverse events. […] Approximately 10–50% of patients after extended TPO-RA exposure are able to taper and eventually discontinue treatment without relapse. […] However, predictors of long-term response after TPO-RA discontinuation are lacking, but progressive tapering may be preferentially attempted in patients with a stable CR.

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