G. Bries MD, PhD

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

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)

BHS 2013 recommendations for treatment of myelodysplastic syndromes

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)

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

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)

Update on the initial therapy of multiple myeloma

Summary

With the introduction of immunomodulatory drugs and proteasome inhibitors, major improvements have been achieved in the treatment and prognosis of multiple myeloma. Different treatment combinations are now in use and innovative therapies are being developed. This rapidly changing therapeutic landscape calls for an update on the Belgian myeloma guidelines, published in 2010.¹ Based on an extensive review of the recent literature, the myeloma study group of the Belgian Hematology Society has revised the consensus recommendations on myeloma care, to be used by haematologists as a reference for daily practice. When applicable, comments with regards to the Belgian reimbursement modalities are included. The full text with appendices can be downloaded from the Belgian Hematology Society website (www.bhs.be) and from the Belgium Journal of Hematology website (www.ariez.com).

(BELG J HEMATOL 2014;5(4):125–36)

P2.18 Responding patients show durable responses to bendamustine in double refractory multiple myeloma patients

P3.05 Efficacy of azacitidine in Belgian patients: results of a real-life non-interventional, post-marketing survey

Primary immune thrombocytopenia in adults

Summary

The Belgian Hematological Society (BHS) guideline panel on adult primary immune thrombocytopenia (ITP) reviewed the recent literature on diagnosis and treatment to make recommendations on the best strategies for frontline and subsequent-line treatment. No treatment is necessary for patients with platelet counts higher than 30000/μl in the absence of bleeding symptoms. Patients newly diagnosed or relapsing after a long-term treatment-free period can be managed with corticosteroids with or without intravenous immunoglobulins. A second line therapy is indicated for those patients who are intolerant or unresponsive to or relapse after initial corticosteroid treatment and have a risk of bleeding. The guideline panel recommends splenectomy as it is the treatment with the highest curative potential and an acceptable safety profile. If possible, splenectomy should be delayed to at least twelve months after diagnosis as spontaneous remission can occur in this time period. Thrombopoietin receptor (TPO-R) agonists are recommended for patients who are refractory to or relapse after splenectomy or who have a contra-indication to splenectomy irrespective of the duration of ITP. The guideline panel agrees that rituximab, azathioprine, cyclophosphamide, cyclosporine A, danazol, dapsone, mycophenolate mofetil and vincristine/vinblastine are potential treatment options, especially for patients refractory to TPO-R agonists.

(BELG J HEMATOL 2013;1:11–20)