Lymphoplasmacytic lymphoma / Waldenstrom’s macroglobulinaemia

Clinical features

§        Monoclonal serum IgM +/- hyperviscosity

§        PP may have autoantibody or cryoglobulin activity

§        Neuropathies

§        IgM and myelin sheath antigen interactions

§        Coagulopathies

§        IgM binding to factor, platelets, fibrin

Aetiology

§        Association of type 2 cryoglobulinaemia and HCV in LPL

§        Reducing viral load has been associated with regression of lymphoma

Morphology

§        Lymph nodes

§        Diffuse without pseudofollicles

§        Interfollicular with sparing of the sinuses

§        May be increased immunoblasts

§        Bone marrow /PB

§        Nodular and/ or diffuse

Immunophenotype

§        Surface and cytoplasmic Ig positive and CD5 negative

§        B markers positive

§        Negative for CD10, CD23

Cytogenetics

§        T(9;14) = PAX-5 gene in up to 50%

Treatment

§        Plasmapheresis for hyperviscosity

§        May not be required

§        Alkylating agents with pred

§        Purine analogues

§        Rituximab

§        Patients resistant to alkylators can be considered for combination with purine analogue and ritux

§        Fludarabine better than COP