Lymphoplasmacytic lymphoma
From IDWiki
Definition
- Low-grade B-cell neoplasm frequently causing an IgM gammopathy (at which point it is called Waldenstrom's macroglobulinemia)
Investigations
- SPEP and immunofixation showing IgM-kappa M-protein
Clinical Presentation
- Hyperviscosity, including neurological symptoms
- Peripheral neuropathy
- Lymphadenopathy, organomegaly, cytopenia
- Constitutional symptoms
Management
- Depends on underlying health and age
- Treatment only indicated if symptoms progressing
- From most to least effective (and most to least side effects):
- RCP: rituximab, cyclophosphamide, prednisone (or dexamethasone for DRC)
- BR: bendamustine/rituximab
- Chlorambucil/Obinutuzumab
- Ibrutinib
- Before chemotherapy, screen for hepatitis B +/- TB/Strongy
