Canterbury DHB

Context

Splenic Marginal Zone Lymphoma

Patients usually present with splenomegaly, which may be massive. Blood and bone marrow involvement is present in 95% of patients. The abnormal cells may have cytoplasmic projections (villous lymphocytes) and the condition needs to be distinguished from hairy cell leukaemia. The cells are positive for surface Ig, CD20 and 79a. The cells are negative for CD5, 10, 23 and 103. Nuclear cyclin D1 is absent. No consistent cytogenetic changes have been noted.

In This Section

Management

Management

Observation. If significant and/or progressive splenic enlargement occurs, treat with rituximab alone, or as for other low grade lymphoma - e.g., BR. The disease responds well to splenectomy.

About this Canterbury DHB document (5988):

Document Owner:

Peter Ganly (see Who's Who)

Issue Date:

November 2018

Next Review:

November 2021

Keywords:

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Topic Code: 5988