Clinical features of indolent lymphomas

Up to 40% of cases; slowly progressive disorders.

Follicular lymphoma: most common in middle and old age (median age 55 years); presents with painless lymphadenopathy at >1 sites, effects of BM infiltration, constitutional symptoms (15-20%) or pressure effects of bulky nodes (ureter, spinal cord or orbit); LN may fluctuate in size; at diagnosis, 66% stage III or IV, 70% BM involvement, 15-20% localised stage I or II disease; median survival —8-10 years; ~30% may transform to high grade DLBCL (often resistant to treatment; median survival 12 months). Note:

cases of FL with a high proportion of centroblasts (>50%) on histology follow a more aggressive clinical course and are treated as aggressive lymphomas.

Marginal zone lymphomas take 3 forms:

• Mucosa-associated lymphoid tissue (MALT) lymphomas—associated with local invasion at site of origin, e.g. stomach, small bowel, salivary gland or lung; gastric MALT lymphomas present with long history of 197 abdominal pain; diagnosis by endoscopic biopsy; localised in 80-90% and respond to antibiotic treatment for H pylori; good prognosis (>80% 5 year survival).

• Nodal marginal zone lymphoma (MZL) or monocytoid B-cell lymphoma rare; associated with Sjogren's syndrome—usually localised to head, neck and parotid gland.

• Spleen MZL related to SLVL (p182); elderly patients with marked splenomegaly ± hypersplenism, BM involvement ± villous lymphocytosis, lymphadenopathy absent.

Small lymphocytic lymphoma: nodal form of CLL (p168); generally age >60 years; disseminated peripheral lymphadenopathy and splenomegaly; lymphocyte count <4.5 x 109/L; BM involvement in 80%; constitutional symptoms <20%; serum paraprotein, usually IgM in 30%; median survival 8-10 years; some patients evolve into CLL, others to DLBCL.

Lymphoplasmacytic lymphoma: occurs in older patients; usually isolated lymphadenopathy ± serum paraprotein; usually IgM, symptoms of hyperviscosity if markedly 4 (^Waldenstrom's macroglobulinaemia, p284).

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