Nodal marginal zone B-cell lymphoma
ORPHA:86867· ICD-10 C83.0
Definition
Nodal marginal zone B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma, characterized by abnormal clonal proliferation of mature B-lymphocytes with involvement of the lymph nodes, sometimes the bone marrow, and rarely the blood. Clinically it presents with disseminated peripheral, abdominal and/or thoracic lymphadenopathy. Cytopenia and bulky tumors (greater than 5 cm) are rare. Association with Hepatitis C virus and chronic inflammation has been reported.
- Prevalence
- 1-9 / 100 000
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly