Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Treatment
This is an indolent lymphoma, which is incurable with
conventional treatment. Patients with a hyperviscosity syndrome
should be treated as an emergency with plasma exchange until
plasma viscosity has normalised. Plasma exchange should be
continued regularly until IgM production has been controlled
sufficiently to prevent further episodes of hyperviscosity.
Oral Chlorambucil and Fludarabine are the most commonly
used agents in LPL, usually for 6 months.
Combination immunochemotherapy can be used as first line
therapy in young patients or as second line therapy.
Combinations include Rituximab with Fludarabine, or
Cycophosphamide. Bortezomib is active in LPL and combining
it with Dexamethasone and Fudarabine results in an ORR
of 96% and CR in over 20% of patients. Vincristine and possibly
anthracyclines may not be active in LPL. Rituximab is active in
LPL but should only be used when the paraprotein is low
(eg after plasma exchange) because of the risk of aggravating
hyperviscosity and a high incidence of severe infusional reactions.
Response Evaluation
Normalisation of blood count, total, reduction of igM with
plateau levels of the protein and a normal viscosity. The viscosity
should be checked weekly until normalisation and then monthly
while on treatment
Follow Up
Two monthly for 1 year, 3 monthly for the second year, followed
by long term follow up between 6 – 12 monthly with FBC,
biochemistry profile, paraprotein level and viscosity.
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