Evaluation and Management of Disease Transformation in Waldenström Macroglobulinemia.
Fiche publication
Date publication
mai 2023
Journal
Hematology/oncology clinics of North America
Auteurs
Membres identifiés du Cancéropôle Est :
Pr DELMER Alain
Tous les auteurs :
Talaulikar D, Tomowiak C, Toussaint E, Morel P, Kapoor P, Castillo JJ, Delmer A, Durot E
Lien Pubmed
Résumé
Histologic transformation (HT) to diffuse large B-cell lymphoma occurs rarely in Waldenström macroglobulinemia, with higher incidence in MYD88 wild-type patients. HT is suspected clinically when rapidly enlarging lymph nodes, elevated lactate dehydrogenase levels, or extranodal disease occur. Histologic assessment is required for diagnosis. HT carries a worse prognosis compared with nontransformed Waldenström macroglobulinemia. A validated prognostic score based on three adverse risk factors stratifies three risk groups. The most common frontline treatment is chemoimmunotherapy, such as R-CHOP. Central nervous system prophylaxis should be considered if feasible and consolidation with autologous transplant should be discussed in fit patients responding to chemoimmunotherapy.
Mots clés
Diffuse large B-cell lymphoma, Histologic transformation, MYD88(L265P) mutation, Waldenström macroglobulinemia
Référence
Hematol Oncol Clin North Am. 2023 05 26;: