C. Doyen MD

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)

A case report of an atypical POEMS syndrome

summary

POEMS syndrome is a rare paraneoplastic syndrome that is defined by the presence of peripheral neuropathy, a monoclonal plasma cell disorder, and other paraneoplastic features, of which the most common include organomegaly, endocrinopathy and skin changes.¹ We report a case of POEMS syndrome in a 62-year old female who presented with worsening general condition, weight loss, asthenia and diarrhoea. Clinical examination showed the presence of ascites, peripheral oedema and a thickened skin with the presence of glomeruloid hemangioma. Further investigations showed the presence of three isolated FDG-avid bone laesions on PET-CT, a plasmacytoma with lambda restriction on bone marrow biopsy and elevated VEGF serum levels. The patient was treated with local radiotherapy with a total dose of 39 gray. Two months after radiotherapy, the patient already has a good clinical response with a reduction of ascites, fluid retention and diarrhoea, associated with a significant decline in the VEGF level.

After the case description, a review of the literature is presented.

(BELG J HEMATOL 2015;6(4): 156–61)

P2.03 Belgian Stop IMatinib trial

Pomalidomide: a new hope for relapsed and refractory multiple myeloma

Summary

Relapsed and refractory multiple myeloma patients have a particularly poor prognosis and the development of new drugs is urgently needed. Pomalidomide, a third-generation immunomodulatory drug with a pleiotropic activity, was approved in 2013 by the Food and Drug Administration and the European Medicines Agency, in association with low dose dexamethasone in relapsed and refractory multiple myeloma, in patients who received at least two prior therapies, including bortezomib and lenalidomide and demonstrated progression on the last therapy. In the phase III MM-003 study, pomalidomide associated with low dose dexamethasone was superior to high dose dexamethasone in these patients, with a manageable safety and tolerability profile. This paper will review the available data concerning the mechanisms of action, the efficacy in clinical studies and the safety of this very promising new drug.

(BELG J HEMATOL 2014;5(4):137–42)

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)

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

Summary

Mantle cell lymphoma was recognised in the nineties and is characterised by the t(11;14)(q13;q32) translocation which results in overexpression of cyclin D1.¹ This disease represents approximately 6% of all non-Hodgkin’s lymphomas. Mantle cell lymphoma generally affects patients over 60 years-old. Most patients have advanced disease (>70 % Ann Arbor stage IV). Several efforts have been made to predict outcome in mantle cell lymphoma. The cell-proliferation marker Ki-67, the Mantle Cell Lymphoma International Prognostic Index, fluorodeoxyglucose positron emission tomography and minimal residual disease are prognostic tools. For young patients, chemoimmunotherapy followed by high-dose chemotherapy plus stem cell transplantation is the treatment of choice. For the main group of older patients, chemo-immunotherapy followed by maintenance with rituximab is the gold standard. In relapses, temsirolimus is actually registered and new drugs, such as ibrutinib, are currently evaluated with promising preliminary results.^2–5

(BELG J HEMATOL 2014;5(3):89–96)

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