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ORPHA:33226Disease
What it is
A rare indolent B-cell non-Hodgkin lymphoma, characterized by the infiltration of monoclonal lymphoplasmacytic cells in the bone marrow and the production of serum immunoglobulin M (IgM) monoclonal protein.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Elderly
- 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.
Signs and symptoms
Common30–79%
7Sometimes5–29%
35- Abnormal retinal vascular morphology
- Anorexia
- Ataxia
- Congestive heart failure
- Cranial nerve paralysis
- Cryoglobulinemia
- Cutis marmorata
- Diarrhea
and 27 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
Cross-references
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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