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Persistent polyclonal B-cell lymphocytosis

ORPHA:300324· ICD-10 D72.8

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.

Prevalence
<1 / 1 000 000
Inheritance
Multigenic/multifactorial
Age of onset
No data available