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Primary mediastinal large B-cell lymphoma

Disease definition

Primary mediastinal B-cell lymphoma (PMBL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL; see this term), arising from B cells of thymic origin, that affects mainly women between the ages of 20-30, that usually presents with a bulky and rapidly expanding anterior mediastinal mass, often with pleural and pericardial effusions, and that can invade the lungs, superior vena cava, pleura, pericardium, and chest wall, leading to manifestations of cough, dyspnea, and superior vena cava syndrome.

ORPHA:98838

Classification level: Disorder
  • Synonym(s):
    • Large cell lymphoma of the mediastinum
    • Med-DLBCL
    • Mediastinal diffuse large-cell lymphoma with sclerosis
    • Primary mediastinal clear cell lymphoma of B-cell type
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: C83.3
  • OMIM: -
  • UMLS: C0855152  C1292754
  • MeSH: -
  • GARD: -
  • MedDRA: 10036710

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