M. Delforge 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)

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)

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)

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)

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)

P4.10 De novo thrombotic microangiopathy after non-renal solid organ transplantation

P3.02 Autologous Hematopoietic Stem Cell Transplantation in Crohn’s Disease: Results from our first 14 patients