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Persistent polyclonal B-cell lymphocytosis

Disease definition

Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare, generally benign, lymphoproliferative hematological disease characterized by: chronic, stable, persistent, polyclonal lymphocytosis of memory B-cell origin, the presence of binucleated lymphocytes in the peripheral blood, and a polyclonal increase in serum immunoglobulin M (IgM). Patients are most frequently asymptomatic or may present with mild splenomegaly.

ORPHA:300324

Classification level: Disorder
  • Synonym(s):
    • PPBL
    • Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes
  • Prevalence: Unknown
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: No data available
  • ICD-10: D72.8
  • OMIM: 606445
  • UMLS: C1847973
  • MeSH: -
  • GARD: -
  • MedDRA: -
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