M. Delforge MD, PhD

O.1 A new frailty scoring in ‘clinically fit’ older patients with malignant hemopathies admitted to receive chemotherapy

Kappa free light chains presenting as an unusual fraction on serum immunofixation electrophoresis

Summary

A 79-year old man presented at the emergency ward of our hospital with symptoms characteristic for multiple myeloma. Although serum capillary zone electrophoresis appeared normal, kappa free light chains were found in serum and in urine by immunofixation electrophoresis. The kappa free light chain fraction detected in the serum, albeit appeared as a polyclonal-like smear with an aberrant localisation in the alpha-2- and beta-regions. Subsequent high resolution gel electrophoresis of the serum suggested the presence of a monoclonal fraction in the alpha-2- and beta-regions, but quantification of the band by densitometry was still not possible. Quantification of free light chains in the patient’s serum by immunonephelometry indicated the presence of an excessive amount of kappa free light chains. However, overestimation of the amount of kappa free light chains by the immunonephelometric method was suspected because of discrepancy with the intensity of the fractions on serum and urine immunofixation electrophoresis.

(BELG J HEMATOL 2016;7(5):194–8)

Highlights in multiple myeloma

Summary

Multiple myeloma (MM) is one of the haematological malignancies with the fastest therapeutic development. Consequently, interesting new data on both preclinical and clinical progress in MM were presented at the 2016 EHA meeting in Copenhagen. We have selected four clinical and one preclinical abstract for discussion in this paper. The choice is based on the importance of the presentations and the impact their results will have on the future treatment landscape in MM.

(BELG J HEMATOL 2016;7(4):130–3)

O.2 RPL5 on 1p22.1 is recurrently deleted in multiple myeloma and its expression is linked to bortezomib response

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)

Use of multiparameter flow cytometry in multiple myeloma and other plasma cell neoplasms

Summary

Flow cytometric immunophenotyping is recommended for diagnosis, classification and monitoring of disease in monoclonal gammopathies. Furthermore, it is a useful diagnostic tool for clinical practice and has various applications, such as its ability to distinguish between normal, reactive and malignant plasma cells, to evaluate the risk of progression from monoclonal gammopathy of undetermined significance to plasma cell myeloma, to provide prognostic information, to evaluate the presence of minimal residual disease and to identify new therapeutic targets. The incorporation of novel therapies in the management of patients diagnosed with plasma cell neoplasms has increased depth and frequency of response, as well as prolonged progression free and overall survival. Along with these improvements in therapeutic strategies, definition of responses to treatment has evolved over time. It was therefore necessary to develop reproducible and sensitive assays for detection and monitoring of minimal residual disease and to define its prognostic value in predicting progression free and overall survival, to allow for consolidation and maintenance therapeutic strategies and to evaluate the efficacy of novel therapies.

(BELG J HEMATOL 2015;6(2):46–53)