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.
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.
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