K. Fostier 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)

The emerging role of monoclonal antibodies in the treatment of multiple myeloma

summary

Monoclonal antibodies have a profound impact on the prognosis and survival of patients with haematological malignancies. In the treatment of multiple myeloma, until recently, results of monoclonal antibodies have been disappointing. The introduction of two novel classes of monoclonal antibodies holds great promise to change this. Daratumumab (and related antibodies) is a monoclonal antibody directed to CD38, an intriguing multifunctional surface protein abundantly expressed on malignant plasma cells and their precursors. Daratumumab displays impressive single agent activity in heavily pretreated multiple myeloma patients and due to its favourable safety profile, this molecule seems to be an excellent accessory companion to known anti-multiple myeloma regimens and also in monotherapy as a maintenance agent. Elotuzumab, to the contrary, is an anti-CS1 monoclonal antibody, which does not show any clinically relevant single agent activity, but when combined with other anti-multiple myeloma drugs appears to greatly enhance their efficacy and can even revert the refractory state to the agents. When these promising results are confirmed in phase III trials, immunotherapy can finally be incorporated in the treatment schedule of newly diagnosed and relapsed/refractory multiple myeloma patients.

(BELG J HEMATOL 2015;6(5):209–15)

P3.10 Kaposi’s Sarcoma: a rare complication reflecting immune suppression after autologous stem cell transplantation

P2.19 Light chain cast nephropathy in a patient with Waldenström’s macroglobulinemia

P2.03 Immunomodulatory Drugs Restore Effector Cell Immune Functions In Myeloma Patients With Low Disease Burden After Autologous Stem Cell Transplantation

Immunomodulatory drugs: new developments

Summary

The immunomodulatory drugs (IMiDs) are a class of orally available compounds which are licensed for the treatment of multiple myeloma (thalidomide, lenalidomide) and transfusion-dependent low- and intermediate-risk myelodysplasia (MDS) with deletion of long arm of chromosome 5 (lenalidomide). Pomalidomide, a novel second generation IMiD, is entering clinical trials and seems to further broaden the therapeutic spectrum of these already pleiotropic drugs. Here we summarise new insights into the mechanism of action of IMiDs as well as new developments related to their clinical use, as maintenance therapy in multiple myeloma (MM), in the treatment of myeloproliferative neoplasm- associated myelofibrosis, other types of MDS, chronic lymphocytic leukaemia (CLL) and sickle cell disease (SCD).

(BELG J HEMATOL 2013;1:21–28)

P.19 Clonaly related monoclonal B-cell lymphocytosis and diffuse large B-cell lymphoma in a patient treated for EBV-positive Hodgkin lymphoma