PRACTICE GUIDELINES

Diagnosis and treatment of AL Amyloidosis in 2015: Consensus guidelines of the Belgian Hematological Society

BJH - volume 6, issue 5, december 2015

A. Kentos MD, B. De Pryck MD, C. Doyen MD, PhD, G. Bries MD, PhD, I. Van de Broek MD, PhD, J. Caers MD, PhD, K. Beel MD, PhD, K. Fostier MD, K.L. Wu MD, PhD, M-C. Vekemans MD, M. Delforge MD, PhD, N. Meuleman MD, PhD, P. Mineur MD

Summary

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 more

Updated BHS guidelines for the treatment of chronic lymphocytic leukaemia anno 2016

BJH - volume 6, issue 5, december 2015

A. Janssens MD, PhD, D. Bron MD, PhD, E. Van den Neste MD, PhD, F. Offner MD, PhD

summary

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 more

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

BJH - volume 6, issue 5, december 2015

A. Janssens MD, PhD, A. Van Hoof MD, PhD, E. Mourin MD, E. Van den Neste MD, PhD, G. Verhoef MD, PhD, M. André MD, V. Vergote MD

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)

Read more

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

BJH - volume 6, issue 4, october 2015

A. Janssens MD, PhD, A. Van Hoof MD, PhD, C. Bonnet MD, PhD, D. Bron MD, PhD, D. Dierickx MD, PhD, E. Van den Neste MD, PhD, G. Verhoef MD, PhD, K.L. Wu MD, PhD, M. André MD, P. Heimann MD, PhD, T. Tousseyn MD, PhD, V. De Wilde MD, PhD, V. Van Hende MD

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)

Read more

BHS 2013 recommendations for treatment of myelodysplastic syndromes

BJH - volume 6, issue 2, may 2015

C. Graux MD, PhD, D. Breems MD, PhD, D. Selleslag MD, G. Bries MD, PhD, L. Noens MD, PhD, M. Delforge MD, PhD, S. Meers MD, PhD

Summary

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 more

BHS Guidelines for the treatment of Burkitt’s lymphoma

BJH - volume 6, issue 2, may 2015

A. Janssens MD, PhD, A. Van Hoof MD, PhD, B. De Prijck MD, C. Bonnet MD, PhD, D. Bron MD, PhD, D. Dierickx MD, PhD, G. Verhoef MD, PhD, K.L. Wu MD, PhD, M. André MD, P. Heimann MD, PhD, T. Tousseyn MD, PhD, V. Van Hende MD, W. Schroyens MD, PhD, Y. Beguin MD, PhD

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)

Read more

The 2013 Belgian Hematological Society recommendations for the diagnosis and classification of myelodysplastic syndromes

BJH - volume 6, issue 1, march 2015

C. Graux MD, PhD, D. Breems MD, PhD, D. Selleslag MD, G. Bries MD, PhD, L. Noens MD, PhD, M. Delforge MD, PhD, S. Meers MD, PhD

Summary

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)

Read more
X