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- Birgegard, Gunnar
(författare)
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Anagrelide treatment in myeloproliferative disorders
- 2006
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Ingår i: Seminars in Thrombosis and Hemostasis. - : Georg Thieme Verlag KG. - 0094-6176 .- 1098-9064. ; 32:3, s. 260-266
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Tidskriftsartikel (refereegranskat)abstract
- Platelet-lowering therapy in myeloproliferative disorders includes cytostatic drugs, mainly hydroxyurea, interferon alpha, and anagrelide. Anagrelide is the latest addition to the therapeutic arsenal, and the basis for its use is reviewed. The platelet-lowering efficacy is 70 to 80% in essential thrombocythemia, and the response is rapid; most of the patients reach the treatment goal within a few weeks. Side effects are common, mainly caused by the vascular effects, and include palpitation, headache, loose stools/diarrhea, and edema. Some side effects are time-limited, but late dropout from therapy is not uncommon. The total dropout rate in prospective studies is 30 to 50%. Pharmacologic treatment of side effects is often helpful. Cardiac insufficiency may be worsened in patients with previous heart failure, and special caution is warranted in such patients. Anagrelide has recently been registered in Europe as a second-line therapy in ET but is often used as first-line therapy in the United States, especially in younger patients, due to the concern about increased leukemia risk with cytostatic treatment. The first randomized anagrelide study, with its limitations, gives support for the second-line registration. Given that dose escalation is a problem in some patients with all therapeutic agents used, combination of two drugs in lower doses is a practical option already used by many clinicians without basis in any published study.
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- Birgegard, Gunnar
(författare)
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Does anything work for anaemia in myelofibrosis?
- 2014
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Ingår i: Baillière's Best Practice & Research. - : Elsevier BV. - 1521-6926 .- 1532-1924. ; 27:2, s. 175-185
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Tidskriftsartikel (refereegranskat)abstract
- Anaemia is a common finding at diagnosis in myelofibrosis, and becomes a symptomatic problem in most patients with time. There are several treatment options for specific anaemia treatment, none of which has been tested in large, randomized, controlled trials. However, as myelofibrosis is not a disease with spontaneous remissions, even non-randomized trials carry weight In this survey, the existing evidence will be analysed, both for the commonly used treatments like erythropoiesis-stimulating agents, androgens and thalidomide and for the new drugs in the area, and conclusions will be drawn concerning standard clinical anaemia treatment in myelofibrosis, which according to evidence from studies has a 40-50% chance of response in patients with not too advanced disease. (C) 2014 Elsevier Ltd. All rights reserved.
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- Geyer, Holly, et al.
(författare)
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Symptomatic Profiles of Patients With Polycythemia Vera : Implications of Inadequately Controlled Disease
- 2016
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Ingår i: Journal of Clinical Oncology. - 0732-183X .- 1527-7755. ; 34:2, s. 151-
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) associated with disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydroxyurea (HU) use (including resistance or intolerance), phlebotomy requirements, and palpable splenomegaly. We aimed to determine how these features contribute alone and in aggregate to the PV symptom burden.PATIENTS AND METHODS: Through prospective evaluation of 1,334 patients with PV who had characterized symptom burden, we assessed patient demographics, laboratory data, and the presence of splenomegaly by disease feature (ie, known HU use, known phlebotomy requirements, splenomegaly).RESULTS: The presence of each feature in itself is associated with a moderately high symptom burden (MPN symptom assessment form [SAF] total symptom score [TSS] range, 27.7 to 29.2) that persists independent of PV risk category. In addition, symptoms incrementally increase in severity with the addition of other features. Patients with PV who had all three features (PV-HUPS) faced the highest total score (MPN-SAF TSS, 32.5) but had similar individual symptom scores to patients with known HU use (PV-HU), known phlebotomy (PV-P), and splenomegaly (PV-S).CONCLUSION: The results of this study suggest that patients with PV who have any one of the features in question (known HU use, known phlebotomy, or splenomegaly) have significant PV-associated symptoms. Furthermore, it demonstrates that many PV symptoms remain severe independent of the number of features present.
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