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Start free with EleplanAutoimmune lymphoproliferative syndrome
ORPHA:3261Disease
Also called ALPS · Canale-Smith syndrome
What it is
A rare, inherited disorder characterized by non-malignant lymphoproliferation, multilineage cytopenias, and a lifelong increased risk of Hodgkin's and non-Hodgkin's lymphoma.
Key facts
- Age of onset
- All ages
- Inheritance
- Autosomal dominant, Autosomal recessive
- Classified as
- Disease
Signs and symptoms
Very common80–99%
4- Autoimmunity
- Chronic noninfectious lymphadenopathyDiagnostic criterion
- Lymphadenopathy
- Splenomegaly
Common30–79%
14- Abnormal bleeding
- Abnormal circulating interleukin concentration
- Abnormal lymphocyte apoptosisDiagnostic criterion
- Autoimmune hemolytic anemiaDiagnostic criterion
- Autoimmune thrombocytopeniaDiagnostic criterion
- Bruising susceptibility
- Elevated proportion of CD4-negative, CD8-negative, alpha-beta regulatory T cellsDiagnostic criterion
- Hepatomegaly
- Hypersplenism
- Increased B cell count
- Increased circulating IgG level
- Increased circulating immunoglobulin concentrationDiagnostic criterion
- Increased circulating interleukin 10 concentrationDiagnostic criterion
- Neutropenia in presence of anti-neutropil antibodiesDiagnostic criterion
Sometimes5–29%
31- Abnormal proportion of CD4 T cells
- Abnormal proportion of CD8 T cells
- Abnormal vitamin B12 levelDiagnostic criterion
- Antineutrophil antibody positivity
- Antinuclear antibody positivity
- Antiphospholipid antibody positivity
- B-cell lymphoma
- Burkitt lymphoma
and 23 more in this range
Rare1–4%
23- Arthritis
- Basal cell carcinoma
- Bone marrow hypercellularity
- Bone marrow hypocellularity
- Colitis
- Fibroadenoma of the breast
- Gastritis
- Headache
and 15 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.
Genes
ICD-10 codes
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
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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