Small lymphocytic lymphoma

This has similar clinical and laboratory features to chronic lymphocytic leukaemia but has no excess of circulating lymphocytes. It is often widespread but indolent and treatment may not be indicated.

Lymphoplasmacytic lymphoma

This is a disease with small lymphocytes, plasma cells and cells with lymphoplasmacytoid appearances in the lymph nodes, spleen and/or bone marrow. There is usually a paraprotein in serum. If this is IgM and >3 g/L, the disease is termed Waldenström macroglobulinaemia. High levels of this protein may be associated with hyperviscosity with visual disturbance, confusion, impaired consciousness and headaches (Fig. 34.1).

Follicular lymphoma

This is the most frequent form of low-grade lymphoma, mainly involving lymph nodes, usually widespread at diagnosis. The spleen and bone marrow are often involved and the patient may present with a primary skin or small intestinal tumour. The histology is characteristic (Fig. 33.3). It is subdivided into three grades (a–c) depending on the proportion of small and large cells in the neoplastic follicles. Over 80% of cells show the t(14;18) translocation with increased expression of BCL-2, which inhibits cell apoptosis.

The clinical course is often indolent for many years with therapy not being needed, but transformation to a more aggressive large cell lymphoma occurs in about one-third of cases when the prognosis is substantially reduced. However, Stage Ia or IIa disease may be cured by local radiotherapy in 50–80% of cases (see Chapter 35).

Marginal cell lymphoma

Splenic marginal cell lymphoma presents with an enlarged spleen, often circulating monoclonal B lymphocytes, autoimmune haemolysis and a paraprotein. Mucosa-associated lymphoid tissue (MALT) lymphomas occur, particularly in the stomach, associated with Helicobacter pylori infection in early stages, and this may respond to antibiotic therapy. The disease may also involve the thyroid, lung or other soft tissues.

Mantle cell lymphoma

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