P. Heimann MD, PhD

P31 Approach of an IgM monoclonal gammopathy: clinical relevance of the MYD88 L265P mutation

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

summary

Waldenström’s macroglobulinaemia is a B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with demonstration of an IgM monoclonal gammopathy in the blood. This condition belongs to the lymphoplasmacytic lymphomas as defined by the World Health Organization classification (ICD-0 code 9671/3). Approximately one-fourth of patients are asymptomatic. Clinical features of the symptomatic patients are diverse and may relate to overall disease burden (such as peripheral blood cytopaenias, organomegaly and constitutional symptoms) or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome, amyloidosis, peripheral neuropathy and cold haemagglutinin. Therapeutic options have traditionally involved alkylating agents, nucleoside analogues, and rituximab, either as single therapy or in combination. However, emerging new data on combination therapy as well as novel agents have shown encouraging results. This report provides the Belgian Hematology Society guidelines according to recent clinical studies.

(BELG J HEMATOL 2015;6(4):142–50)

BHS Guidelines for the treatment of Burkitt’s lymphoma

Summary

Burkitt’s lymphoma is a rare but very aggressive non-Hodgkin’s lymphoma characterised by an isolated translocation t(8;14)(q24;q32). The sporadic form is the sub-entity most frequently encountered in Belgium. Diagnosis and initial work-up must be completed rapidly to start treatment as soon as possible. Positron emission tomography scan is useful for initial staging and to evaluate the chemosensitivity of the tumour during and after treatment. After debulking, it is recommended to add rituximab to chemotherapy. Currently intensive short-cycle and low intensity chemotherapies are two valuable options. Radiotherapy is not indicated except in case of central nervous system involvement. Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured. For patients in partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies.

(BELG J HEMATOL 2015;6(2):61–9)