Persistent polyclonal B-cell lymphocytosis

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Persistent polyclonal B-cell lymphocytosis

ORPHA:300324Disease

Also called PPBL · Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes

What it is

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.

Key facts

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

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

CARD11Major susceptibility factor

ICD-10 codes

D72.8filed under a broader ICD-10 category — shared with 4 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MESH C564707MONDO 0011672OMIM 606445UMLS C1847973

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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