Waldenström Macroglobulinaemia is a rare indolent B-cell lymphoproliferative disorder, which is characterised by infiltration of the bone marrow by lymphoplasmacytic cells in combination with a monoclonal IgM gammopathy. In 2015, the first Belgian guidelines on diagnosis, staging and treatment were published with a first update in 2018. Since then, however a lot has changed. The discovery of the molecular markers MYD88 affected the diagnostic work up. There have also been radical changes in treatment and prognosis due to the use of Bruton tyrosine kinase inhibitors. In this second update, we revise both diagnosis and treatment and we have a look at the future.

(BELG J HEMATOL 2023;14(3):122–34)