Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Potential Pitfalls
■ Failure to establish a diagnosis in the patient presenting
with isolated, unexplained splenomegaly – the decision
to proceed with diagnostic splenectomy may be difficult,
especially in elderly patients.
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■
Failure to distinguish from other CD5-negative indolent
B cell lymphomas.
Failure to distinguish from nodal marginal zone lymphoma.
Treatment
Most behave in an indolent fashion, and patients should be
treated as for low-grade/follicular lymphoma. Median survival is
10-13 years. Splenectomy may be followed by haematological
responses and prolonged survival, and is the treatment of choice
for fit patients. Other treatment options include splenic irradiation,
alkylating agents, purine analogues or anti-CD20 antibody.
Transformation to large cell lymphoma may occur.
Response Evaluation and Follow Up
As for other indolent lymphomas.
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