BJH - volume 6, issue 5, december 2015
A. Janssens MD, PhD, E. Van den Neste MD, PhD, F. Offner MD, PhD, D. Bron MD, PhD
The Belgian Hematological Society Lymphoproliferative Working Party updated the 2012 recommendations on the best strategies for front-line and subsequent-line treatment of small lymphocytic leukaemia/chronic lymphocytic leukaemia. No treatment is necessary for patients without active and/or advanced disease, regardless of prognostic factors. When front-line treatment is indicated we recommend adding an anti-CD20 monoclonal antibody to chemotherapy except in frail patients: fludarabine, cyclophosphamide, rituximab for fit patients; bendamustine, rituximab for fit patients >65 years or unfit for fludarabine, cyclophosphamide, rituximab; and chlorambucil with obinutuzumab or rituximab for older patients with a geriatric profile, major comorbidities or a reduced performance status. The choice of treatment for patients with recurrent disease depends on the duration of response to the previous treatment, the type of treatment refractoriness and the presence of a 17p deletion/p53 mutation. As an alternative, chemoimmunotherapy can be proposed for patients with a late relapse. The novel B-cell receptor inhibitors are the best choice for those relapsing early, who have refractory disease or are unfit for chemoimmunotherapy. The B-cell receptor inhibitors are also first choice for each patient with a de novo or acquired 17p deletion/p53 mutation. Reduced intensity conditioning allogeneic stem cell transplantation should still be considered for patients with high-risk disease after response induction by the B-cell receptor inhibitors. We still have to encourage patients to enter clinical trials exploring new drug combinations.
(BELG J HEMATOL 2015;6(5): 195–202)
Read moreBJH - volume 6, issue 5, december 2015
K. Beel MD, PhD, M-C. Vekemans MD, G. Bries MD, PhD, J. Caers MD, PhD, B. De Pryck MD, K. Fostier MD, A. Kentos MD, PhD, N. Meuleman MD, PhD, P. Mineur MD, I. Van de Broek MD, PhD, K.L. Wu MD, PhD, C. Doyen MD, PhD, M. Delforge MD, PhD
Immunoglobulin light chain amyloidosis is a clonal plasma cell dyscrasia, historically associated with a very poor prognosis. Prompt diagnosis is critical to preserve organ function and improve survival in immunoglobulin light chain amyloidosis patients. The severity of cardiac involvement and response to treatment are the most important prognostic factors. Serum free light chain ratio and cardiac biomarkers troponin T and N-terminal pro-brain natriuretic peptide are powerful tools for the evaluation of prognosis and treatment response. Historically, treatment with autologous stem cell transplantation appears to offer a survival benefit, but is only an option in a minority of patients. IMiDs, and especially proteasome inhibitors, have shown promising activity in immunoglobulin light chain amyloidosis. Supportive care should be integrated in the treatment plan and requires a multidisciplinary approach. These guidelines summarise a consensus of the myeloma subcommittee of the Belgian Hematological Society on diagnosis, cytoreductive and supportive treatment of immunoglobulin light chain amyloidosis, based on an extended review of the literature. Where applicable, comments were added with respect to the Belgian reimbursement modalities.
(BELG J HEMATOL 2015;6(5):187–94)
Read moreBJH - volume 6, issue 4, october 2015
V. Van Hende MD, D. Bron MD, PhD, E. Van den Neste MD, PhD, C. Bonnet MD, PhD, M. André MD, PhD, A. Van Hoof MD, PhD, D. Dierickx MD, PhD, G. Verhoef MD, PhD, T. Tousseyn MD, PhD, A. Janssens MD, PhD, V. De Wilde MD, PhD, K.L. Wu MD, PhD, P. Heimann MD, PhD
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)
Read moreBJH - volume 6, issue 2, may 2015
C. Bonnet MD, PhD, A. Janssens MD, PhD, K.L. Wu MD, PhD, W. Schroyens MD, PhD, V. Van Hende MD, P. Heimann MD, PhD, T. Tousseyn MD, PhD, M. André MD, PhD, D. Bron MD, PhD, A. Van Hoof MD, PhD, G. Verhoef MD, PhD, B. De Prijck MD, Y. Beguin MD, PhD, D. Dierickx MD, PhD
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)
Read moreBJH - volume 6, issue 2, may 2015
S. Meers MD, PhD, D. Breems MD, PhD, G. Bries MD, PhD, M. Delforge MD, PhD, C. Graux MD, PhD, D. Selleslag MD, L. Noens MD, PhD
The guidelines on the current state-of-the-art in the diagnosis and treatment of myelodysplastic syndromes of the Belgian Hematological Society working group on myelodysplastic syndromes were published in 2013.1 The key points of these guidelines are presented in two issues of the Belgian Journal of Hematology. In this paper we present the optimal treatment of patients with myelodysplastic syndromes within the current limitations of Belgian reimbursement modalities.
(BELG J HEMATOL 2015;6(2):54–60)
Read moreBJH - 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
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)
Read moreBJH - volume 6, issue 1, march 2015
S. Meers MD, PhD, D. Breems MD, PhD, G. Bries MD, PhD, M. Delforge MD, PhD, C. Graux MD, PhD, D. Selleslag MD, L. Noens MD, PhD
The Belgian Hematological Society working group on myelodysplastic syndromes published their guidelines on the state of the art in diagnosis and treatment of myelodysplastic syndromes and the key points of these recommendations are presented in two issues of the Belgian Journal of Hematology.1 In this first paper we present the requirements for a correct diagnosis and classification of patients with myelodysplasia.
(BELG J HEMATOL 2015;6(1):10–5)
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