G. Verhoef MD, PhD

P3.02 Ponatinib as bridge to haematopoietic stem cell transplantation in a patient with a myeloid/lymphoid neoplasm with eosinophilia and abnormality of FGFR1: a case report

P4.10 The Ponatinib Named Patient Programme (NPP): Real-life Data from Belgian Patients

Treatment of mantle cell lymphomas: Updated recommendations of the Belgian Hematological Society 2015

summary

Mantle cell lymphoma is a rare B-cell non-Hodgkin’s lymphoma characterised by a t(11;14) translocation resulting in overexpression of cyclin D1 and cell cycle dysregulation. Mantle cell lymphoma represents approximately 7–9% of all lymphomas in Europe.¹ Although new treatment regimens have improved the outcomes over the last decades, mantle cell lymphoma is still considered one of the worst prognosis B-cell non-Hodgkin’s lymphoma with a median overall survival of less than five years.² In September 2014 the Belgian Hematological Society recommendations for the treatment of mantle cell lymphoma were published.³ Since then, novel therapies such as ibrutinib and bortezomib have been approved by the European Medicines Agency in the treatment of mantle cell lymphoma. We present the new updated recommendations of the Belgian Hematological Society Lymphoproliferative Working Party. For young patients, the first line therapy remains an AraC-containing chemo-immunotherapy followed by high dose chemotherapy and autologous stem cell transplantation. For the main group of elderly patients, chemo-immunotherapy followed by maintenance with rituximab appears to be the gold standard. In relapse we can recommend treatment with BTK-inhibitor ibrutinib as first choice. Temsirolimus is reimbursed as third line treatment. Relapse patients should also be considered for allogeneic stem cell transplantation if eligible.

(BELG J HEMATOL 2015;6(5):203–8)

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

summary

Waldenström’s macroglobulinaemia is a B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with demonstration of an IgM monoclonal gammopathy in the blood. This condition belongs to the lymphoplasmacytic lymphomas as defined by the World Health Organization classification (ICD-0 code 9671/3). Approximately one-fourth of patients are asymptomatic. Clinical features of the symptomatic patients are diverse and may relate to overall disease burden (such as peripheral blood cytopaenias, organomegaly and constitutional symptoms) or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome, amyloidosis, peripheral neuropathy and cold haemagglutinin. Therapeutic options have traditionally involved alkylating agents, nucleoside analogues, and rituximab, either as single therapy or in combination. However, emerging new data on combination therapy as well as novel agents have shown encouraging results. This report provides the Belgian Hematology Society guidelines according to recent clinical studies.

(BELG J HEMATOL 2015;6(4):142–50)

BHS Guidelines for the treatment of Burkitt’s lymphoma

Summary

Burkitt’s lymphoma is a rare but very aggressive non-Hodgkin’s lymphoma characterised by an isolated translocation t(8;14)(q24;q32). The sporadic form is the sub-entity most frequently encountered in Belgium. Diagnosis and initial work-up must be completed rapidly to start treatment as soon as possible. Positron emission tomography scan is useful for initial staging and to evaluate the chemosensitivity of the tumour during and after treatment. After debulking, it is recommended to add rituximab to chemotherapy. Currently intensive short-cycle and low intensity chemotherapies are two valuable options. Radiotherapy is not indicated except in case of central nervous system involvement. Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured. For patients in partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies.

(BELG J HEMATOL 2015;6(2):61–9)

Practical management of chronic myeloid leukaemia in Belgium

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)

P1.12 Tumor lysis syndrome: monocentric evaluation of a predictive tool