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Cutaneous Marginal Zone Lymphoma : Intro & Clinical

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Cutaneous Marginal Zone Lymphoma (CMZL) - Intro & Clinical : Primary CMZL is a low-grade B-cell lymphoma that makes up about 7% of all cutaneous lymphomas, 30% of primary cutaneous B-cell lymphomas, and about 11% of all extranodal marginal zone lymphomas (MALT lymphomas).

Some of them were previously diagnosed as primary cutaneous immunocytoma, cutaneous lymphoid hyperplasia with monotypic plasma cells, or extramedullary plasmacytoma of skin.

They affect a wide age range; the median age at diagnosis is 50 years. They are slightly more common in males; however, there are conflicting reports regarding sex predominance.

The lesions can be solitary or multiple (more common) and mainly affect trunk (especially back) and arms. They appear as reddish brown/purple/violaceous papules, nodules, or plaques. Ulceration is rare. B-symptoms are not present. CMZL are localized to the skin at presentation (Stage IE). Extracutaneous spread is seen in 4%-8% of patients. Bone marrow involvement is rare and bone marrow biopsy is not routinely recommended.

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