Articles

Management of polycythemia vera: Recommendations from the BHS MPN subcommittee anno 2021

BJH - volume 12, issue 6, october 2021

C. Schuermans MD, D. Mazure MD, K. Van Eygen MD, L. Van Aelst MD, PhD, S. Benghiat Fleur MD, PhD, T. Devos MD, PhD

SUMMARY

Polycythemia vera (PV) is classified by the World Health Organization (WHO) under the BCR-ABL-negative myeloproliferative neoplasms (MPNs) and is characterised by clonal proliferation of myeloid cells, which leads primarily to an increased red blood cell mass. Bone marrow morphology remains the cornerstone of diagnosis. Patients can present with thrombosis, microcirculatory symptoms, haemorrhage, splenomegaly, pruritus and other symptoms that reduce their quality of life and they are at risk of transformation to secondary myelofibrosis (MF) or acute myeloid leukaemia (AML). The main goal of therapy in PV is to minimise the thrombotic risk. To achieve this goal PV patients are being treated with low-dose aspirin and phlebotomies to reach a target haematocrit below 45%. In addition, high-risk patients are being treated with cytoreductive agents. Over the last years, new insights in the pathophysiology, diagnosis and prognosis of polycythemia vera were acquired and novel therapeutic options are available. In this paper we give an update on PV and provide diagnostic and therapeutic recommendations, taking into account the Belgian situation.

(BELG J HEMATOL 2021;12(6):258-74)

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Discontinuation of imatinib in Belgian patients with chronic myeloid leukaemia

BJH - volume 7, issue 5, october 2016

P. Mineur MD, C. Doyen MD, PhD, N. Straetmans MD, PhD, K. Van Eygen MD, D. Pranger MD, A. Bosly MD, PhD, M. André MD, PhD, T. Devos MD, PhD, L. Knoops MD, PhD, On behalf of the MPN Belgian Hematological Society subcommittee

Summary

This article describes the Belgian register of chronic myeloid leukaemia patients who have stopped their treatment with imatinib in conditions comparable to the French STIM trial results: 44% remained in major molecular response off therapy; relapses appear rapidly after stopping imatinib and are responsive when the treatment is resumed.

(BELG J HEMATOL 2016;7(5):184–6)

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Practical management of chronic myeloid leukaemia in Belgium

BJH - volume 6, issue 1, march 2015

F. S. Benghiat MD, PhD, Y. Beguin MD, PhD, B. Dessars MD, PhD, T. Devos MD, PhD, P. Lewalle MD, PhD, P. Mineur MD, N. Straetmans MD, PhD, K. Van Eygen MD, G. Verhoef MD, PhD, L. Knoops MD, PhD

Summary

Imatinib has drastically changed the outcome of patients with chronic myeloid leukaemia, with the majority of them showing a normal life span. Recently, the development of second and third generation tyrosine kinase inhibitors and the possibility of treatment discontinuation made the management of these patients more challenging. In this review, practical management guidelines of chronic myeloid leukaemia are presented adapted to the Belgian situation in 2014. In first line chronic phase patients, imatinib, nilotinib and dasatinib can be prescribed. While second generation tyrosine kinase inhibitors give faster and deeper responses, their impact on long-term survival remain to be determined. The choice of the tyrosine kinase inhibitor depends on chronic myeloid leukaemia risk score, priority for a deep response to allow a treatment-free remission protocol, age, presence of comorbid conditions, side effect profile, drug interactions, compliance concerns and price. Monitoring the response has to be done according the 2013 European LeukemiaNet criteria, and is based on the bone-marrow cytogenetic response during the first months and on the blood molecular response. Molecular follow-up is sufficient in patients with a complete cytogenetic response. For patients who fail frontline therapy, nilotinib, dasatinib, bosutinib and ponatinib are an option depending on the type of intolerance or resistance. T315I patients are only sensitive to ponatinib, which has to be carefully handled due to cardiovascular toxicity. Advanced phase diseases are more difficult to handle, with treatments including allogeneic stem cell transplantation, which is also an option for patients failing at least two tyrosine kinase inhibitors. The possibility of treatment-free remission and pregnancy are also discussed.

(BELG J HEMATOL 2015;6(1): 16–32)

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P2.03 Belgian Stop IMatinib trial

BJH - volume 6, issue Abstract Book BHS, january 2015

P. Mineur MD, C. Doyen MD, PhD, N. Straetmans MD, PhD, K. Van Eygen MD, D. Pranger MD, A. Bosly MD, PhD, M. André MD, PhD, T. Devos MD, PhD, L. Knoops MD, PhD

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P2.07 MPN with JAK2 V617F mutation and identical del20q11.2 in three first-degree relatives without identified germline defect

BJH - volume 6, issue Abstract Book BHS, january 2015

J. Dauw , E. Boone PhD, N. Cardinaels MD, E. Moureau , A. Nijs , F. Nollet PhD, MSc, V. Van Hende MD, N. Van Roy PhD, K. Van Eygen MD

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