United Kingdom immune thrombocytopenia registry: retrospective evaluation of bone marrow fibrosis in adult patients with primary immune thrombocytopenia and correlation with clinical findings

Rizvi, Hasan; Butler, Tom; Calaminici, Mariarita; Doobaree, Indraraj Umesh; Nandigam, Raghava; Bennett, Dimitri; Newland, Adrian C.

doi:10.1111/bjh.13330

Cited by 23 publications

(20 citation statements)

References 13 publications

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“…Whether the risk of BM fibrosis is due to the TPO-RAs themselves or the underlying disease remains unclear. Even in the absence of TPO-RAs, the results of several studies have revealed the presence of background BM reticulin fibrosis in patients with a diagnosis of ITP [6,11,18,19,20]. Furthermore, increased BM reticulin formation has been described in patients with refractory ITP, as well as in those with other autoimmune diseases [21,22].…”

Section: Discussionmentioning

confidence: 99%

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A 2-Year, Longitudinal, Prospective Study of the Effects of Eltrombopag on Bone Marrow in Patients with Chronic Immune Thrombocytopenia

et al. 2016

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Background: The long-term effects of eltrombopag on bone marrow (BM) reticulin and/or collagen deposition in previously treated adults with chronic immune thrombocytopenia (ITP) were assessed. Methods: Three BM biopsies were collected at baseline and after 1 and 2 years of eltrombopag treatment. Specimens were centrally processed, stained for reticulin and collagen, independently reviewed by 2 hematopathologists, and rated according to the European Consensus 0-3 scale of marrow fibrosis (MF). Results: Of 162 patients enrolled, 93 completed all 3 protocol-specified BM biopsies. All patients with a baseline assessment were negative for collagen. Of 159 patients assessed at baseline, 150 (94%) had normal reticulin (MF-0) and 9 (6%) had minimally increased reticulin (MF-1). After 2 years, 83/93 patients (89%) with BM biopsies had MF-0, 10 (11%) had MF-1, and none had MF-2 or MF-3. Five out of 127 patients (4%) at 1 year and 1 out of 93 (1%) at 2 years had collagen deposition. None of the patients had clinical symptoms typical of BM dysfunction or abnormalities of clinical concern based on white blood cell count or peripheral blood smear. Conclusion: For most patients with chronic ITP, eltrombopag is not associated with clinically relevant increases in BM reticulin or collagen formation.

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Section: Discussionmentioning

confidence: 99%

“…Mild reticulin has been shown to be present in the bone marrow (BM) of approximately 27-31% of hematologically normal patients [3,4] and 28-52% of patients diagnosed with primary ITP [5,6]. …”

Section: Introductionmentioning

confidence: 99%

A 2-Year, Longitudinal, Prospective Study of the Effects of Eltrombopag on Bone Marrow in Patients with Chronic Immune Thrombocytopenia

et al. 2016

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“…Thus, a history of thrombosis does not definitely contraindicate TPO-RAs if the platelet count is sufficient to maintain antiplatelet or anticoagulation therapy. Some questions remain about the long-term safety and potential risk of reticulin deposits within the bone marrow with TPO-RAs, but reassuring data have been reported Rizvi et al, 2015). To our knowledge, there are no data to show that reticulin deposits increase with age.…”

Section: Thrombopoietin-receptor Agonists (Tpo-ras)mentioning

confidence: 96%

How we manage immune thrombocytopenia in the elderly

2016

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SummaryWith prolonged life expectancy, immune thrombocytopenia (ITP) is frequent in elderly people. In this setting, ITP diagnosis is challenging because of the concern about an underlying myelodysplastic syndrome. Studies of older adults are lacking, and recommendations for treatment are based mainly on expert opinion. The therapeutic strategy differs from that for younger patients and must take into account the greater risk of bleeding and thrombosis, presence of comorbidities, possible impaired cognitive performance or poor life expectancy and concomitant medications, such as anticoagulant and antiplatelet therapy. Steroids and intravenous immunoglobulin (IVIg) therapy remain the first-line treatments in elderly patients, but prolonged treatment with steroids should be avoided and IVIg treatment may lead to renal failure. Splenectomy is less effective than in young patients and risk of thrombosis is increased. Severe co-morbidities can also contraindicate surgery. Therefore, other second-line treatments are frequently preferred. Danazol and dapsone can be an option for the less severe ITP form. Rituximab is a good option except in patients with a history of infection or with hypogammaglobulinaemia. Thrombopoietin agonists are attractive, especially for patients with severe comorbidities or with limited life expectancy but the risk of thrombosis is a concern.

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“…Moreover, an indirect comparison between patients who received TPOs for 24-26 weeks has demonstrated an overall durable response rate of 42% for eltrombopag and 49% for romiplostim [25]. More importantly, the increase of bone marrow fibrosis appears to be higher (20-31%) than has been suggested [26,27]. …”

Section: Discussionmentioning

confidence: 99%

Thrombopoietin Receptor Agonists Are Often Ineffective in Immune Thrombocytopenia and/or Cause Adverse Reactions: Results from One Hand

Depré

Aboud

Ringel

et al. 2016

Transfus Med Hemother

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Background: Eltrombopag and romiplostim are thrombopoietin receptor agonists (TPOs) that have been increasingly used for the treatment of immune thrombocytopenia (ITP). Based on our experience, the incidence of abortive treatment with these drugs and the occurrence of adverse reactions that lead to therapy break-off despite response are higher than has been previously suggested. Methods: During the last 8 years, a total of 65 patients were treated with eltrombopag and/or romiplostim at our institute. Results: 36 of a total of 58 patients responded well to eltrombopag. In 12 patients that responded, treatment with eltrombopag was discontinued due to the development of adverse reactions. Eltrombopag was replaced by romiplostim in 23 cases (14 non-responders, 9 patients with adverse reactions). Of these patients, 83% responded to romiplostim. Among all patients treated with romiplostim (n = 32), 75% initially responded; however, 8 of these patients developed adverse reactions. Romiplostim was replaced by eltrombopag in 5 cases (4 due to adverse reactions, 1 non-responsive patient), and only 3 (60%) of these patients were observed to respond to eltrombopag. Conclusion: TPOs often remain ineffective in ITP or result in adverse reactions, which lead to treatment stop or to drug switch. Therefore, alternative treatment options are required.

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United Kingdom immune thrombocytopenia registry: retrospective evaluation of bone marrow fibrosis in adult patients with primary immune thrombocytopenia and correlation with clinical findings

Cited by 23 publications

References 13 publications

A 2-Year, Longitudinal, Prospective Study of the Effects of Eltrombopag on Bone Marrow in Patients with Chronic Immune Thrombocytopenia

A 2-Year, Longitudinal, Prospective Study of the Effects of Eltrombopag on Bone Marrow in Patients with Chronic Immune Thrombocytopenia

How we manage immune thrombocytopenia in the elderly

Thrombopoietin Receptor Agonists Are Often Ineffective in Immune Thrombocytopenia and/or Cause Adverse Reactions: Results from One Hand

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