Marginal zone lymphoma

Clinical Young adults and adults; cases in children reported. Solitary or grouped papules or small nodules. Preferential locations: upper extremities, trunk.

Morphology Patchy, nodular or diffuse infiltrates.

Characteristic pattern with central nodular dark area composed of small reactive lymphocytes with or without formation of germinal centres, surrounded by a pale area where neoplastic marginal zone cells and plasma cells predominate.


+ (monoclonal)

Genetics Monoclonal rearrangement of the JH gene detected in 50-60% of cases. t(14;18)(q32;q21) in a minority of cases.

Treatment Excision of solitary lesions; systemic steroids; guidelines radiotherapy; 'watchful waiting'.

Interferon-a and anti-CD20 antibody (rituximab) are effective. Antibiotic treatment may be effective. Systemic chemotherapy reserved for patients with extracutaneous spread.

Fig. 10.9 Immunocytoma. Large dome-shaped tumour on the lower leg.
Fig. 10.10 Immunocytoma. Miliary lesions on the upper leg.
Fig. 10.11 Immunocytoma. (a) Large dome-shaped tumour on the buttock. (b) Note resolution with anetoderma after radiotherapy.

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