Characteristics and management of primary and other immune thrombocytopenias: Spanish registry study

Palau, Javier; Sancho, Esther; Herrera, Magdalena; Sánchez, Silvia Barrientos; Mingot, María Eva; Upegui, Rosa Isabel; Salazar, M. J. Rodríguez; Cruz, Fátima De la; Fernández, Ma. Cristina Lindner; López, Tomás José González; Hernández, José Julio; Ríos, Eduardo; López-Fernández, M. Fernanda; García, Marta; Hernández, José-Ángel; Sanz, Miguel Á.

doi:10.1080/10245332.2017.1311442

Cited by 15 publications

(31 citation statements)

References 32 publications

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“…Whereas ITP is present in up to 30% of cases of systemic lupus erythematosus (SLE), 3 SLE is diagnosed in 2.0%-3.7% of patients with ITP. 4,16,17 As expected, systemic lupus erythematosus (SLE) was the most frequent diagnosis in our series, followed by undifferentiated connective tissue disease. Our patients responded to specific AD treatment, even though two of them also needed eltrombopag to increase their platelet level.…”

Section: Discussionsupporting

confidence: 81%

“…Immune thrombocytopenia is common in AD. Whereas ITP is present in up to 30% of cases of systemic lupus erythematosus (SLE), 3 SLE is diagnosed in 2.0%–3.7% of patients with ITP 4,16,17 . As expected, systemic lupus erythematosus (SLE) was the most frequent diagnosis in our series, followed by undifferentiated connective tissue disease.…”

Section: Discussionsupporting

confidence: 76%

“…1 Around 80%-90% of adult ITP cases are considered primary. 3,4 Secondary ITP cases are a group of immune-mediated thrombocytopenia associated with coexisting conditions as underlying autoimmune diseases (AD), immunodeficiencies (ID), viral infections, vaccine administration or drug exposure. 3,5 Secondary ITP has a complex aetiology as its various forms have different immunologic underpinnings.…”

Section: Introductionmentioning

confidence: 99%

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Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres

et al. 2021

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Aim: To evaluate the incidence and outcome of secondary immune thrombocytopenia (ITP) in a large cohort of paediatric Spanish patients. Methods: A retrospective observational study was conducted in two paediatricUniversity hospitals in Spain between 2009 and 2019, which included children from 4 months to 18 years old diagnosed with ITP. Data were recorded from clinical charts: gender, age at diagnosis, coexisting condition and associated characteristics, outcome and treatment.Results: Secondary ITP was diagnosed in 87 out of 442 patients (19.6%). Postimmunisation ITP was seen in younger children. The onset of secondary ITP to autoimmune diseases (AD) and immunodeficiencies (ID) was at an older age and had more tendency to be insidious, and platelet level was higher than primary ITP. Mean time from ITP onset to AD diseases or ID diagnosis was 1.2 and 2.6 years, respectively. Whereas the cumulative incidence of remission was significantly higher in postimmunisation and post-viral infection (compared with primary ITP patients), it was worse in AD and ID patients.Conclusions: Identification of secondary ITP is important as it predicts outcome. Most of them are diagnosed at ITP onset, but AD diseases and ID should be ruled out periodically as they are usually identified later.

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Section: Discussionsupporting

confidence: 81%

Section: Discussionsupporting

confidence: 76%

Section: Introductionmentioning

confidence: 99%

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Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres

et al. 2021

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“…A long-term treatment period does not improve response rate and should be avoided due to significant adverse events such as infections, high blood sugar, weight gain, and osteoporosis [6]. Despite these known risks, the duration of steroid treatment is often too long [12], and to a large extent, this was also seen in the patients of our study. Nearly a third of the patients treated with steroids received them for more than a year during first-line treatment and this high percentage of patients was carried forward in the subsequent lines.…”

Section: Discussionmentioning

confidence: 64%

Disease management of patients with immune thrombocytopenia—results of a representative retrospective survey in Germany

Kubasch

Kisro²,

Heßling

et al. 2020

Ann Hematol

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Clinical research has resulted in an improvement of treatment options for patients with immune thrombocytopenia (ITP) over the last years. However, only few data exist on the real-life management of patients with ITP. To expand the knowledge, a multicenter, national survey was undertaken in 26 hematology practices distributed all over Germany. All patients with a diagnosis of ITP were documented using questionnaires, irrespective of the diagnosis date over a period of 2 years. Overall, data of 1023 patients were evaluated with 56% of patients being older than 60 years. Seventy-nine percent of the patients had chronic (> 12 months), 16% persistent (> 3–12 months), and 5% newly diagnosed (0–3 months) ITP. In 61% of cases, the disease lasted 3 or more years before survey documentation started. Main strategies applied as first-line therapy consisted of steroids in 45% and a “watch and wait” approach in 41% of patients. During second- and third-line strategies, treatment with steroids decreased (36% and 28%, respectively), while treatment modalities such as TPO-RAs increased (19% and 26%, respectively). As expected, patients with a low platelet count and thus a higher risk for bleeding and mortality received treatment (esp. steroids) more frequently during first line than those with a higher platelet count. Up to a third of patients were treated with steroids for more than a year. Overall, our study provides a cross-section overview about the current therapeutic treatment landscape in German ITP patients. The results will help to improve therapeutic management of ITP patients.

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“…The reported ITP relapse rates are greatly variable among series. 5–7,15–17 For the sample size calculation, a recurrent rate without treatment was estimated as two-thirds (66%) of the patients. There has been no previous report on the effects of low-dose corticosteroid maintenance.…”

Section: Methodsmentioning

confidence: 99%

A Randomized Multicenter Trial Comparing Low-Dose Prednisolone Versus Observation for Prevention of Recurrences in Adult Immune Thrombocytopenia

Pirunsarn

Kijrattanakul

Chamnanchanunt

et al. 2018

Clin Appl Thromb Hemost

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Adult immune thrombocytopenia (ITP) commonly relapses after stopping treatments. This may be preventable by low-dose steroids. In this multicenter study, adult patients with ITP who had been responding to corticosteroids were randomized with the 2 strata of newly diagnosed and relapsed ITP to prednisolone 7.5 mg/d or observation for 6 months. Relapses were defined by a platelet count below 30 × 10/L and/or clinical bleeding. There were 75 patients evaluable for the efficacy and 77 for safety. The recurrent ITP comprised 57.3%. During the median follow-up of 42 weeks, there were 20.5% (8/39) and 25% (9/36) of recurrences in the prednisolone and control groups ( P = .643), with the hazard ratio (HR) of 0.75 ( P = .549). The significant factor that could predict recurrences was relapsed ITP with the HR of 2.79 (95% confidence interval, 1.02-7.64, P = .037). Prednisolone showed a trend toward a benefit in the relapsed subgroup ( P = .070). Adverse events were not different ( P = .540) and mostly mild. In conclusion, prednisolone maintenance could not prolong relapse-free survival. Relapsed patients deserve further investigations for preventive measures.

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Characteristics and management of primary and other immune thrombocytopenias: Spanish registry study

Cited by 15 publications

References 32 publications

Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres

Secondary immune thrombocytopenia in children: Characteristics and outcome of a large cohort from two Spanish centres

Disease management of patients with immune thrombocytopenia—results of a representative retrospective survey in Germany

A Randomized Multicenter Trial Comparing Low-Dose Prednisolone Versus Observation for Prevention of Recurrences in Adult Immune Thrombocytopenia

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