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Plasmablastic lymphoma

Disease definition

A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor.

ORPHA:289666

Classification level: Disorder
  • Synonym(s):
    • PBL
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: C83.3
  • ICD-11: 2A81.2
  • OMIM: -
  • UMLS: C3472614
  • MeSH: -
  • GARD: 12125
  • MedDRA: 10065039
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