Lymphoplasmacytic lymphoma

Definition

• Low-grade B-cell neoplasm frequently causing an IgM gammopathy (at which point it is called Waldenström macroglobulinemia)

Investigations

• SPEP and immunofixation showing IgM-kappa M-protein

Clinical Manifestations

• 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/Strongyloides