Primary cutaneous marginal zone B-cell lymphoma

Orpha code: 178536OMIM code:

Definition

A rare, indolent primary cutaneous B-cell lymphoma characterized by multifocal, red to violaceous papules, plaques or nodules localized predominantly on the trunk and extremities. Histologically, these are dermis infiltrates consisting of small, marginal zone B cells, lymphoplasmacytic cells, and plasma cells. Marginal zone B cells express CD20, CD79a and Bcl-2, and are negative for CD5, CD10 and Bcl-6. Plasma cells are typically located at the periphery, and express CD138, CD79a, and monotypic light chains.

Disease data
Classification

Disease

Synonyms
PCMZL
PCMZL
ORPHA code
178536
OMIM code
-
ICD10 code
C83.0
ICD11 code
2A85.2

No additional description.

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