This neoplasm is not reportable.

Name

Autoimmune lymphoproliferative syndrome (ALP)

Reportability

This neoplasm is not reportable

Abstractor Notes

Autoimmune lymphoproliferative syndrome (ALP) is part of the Tumor like lesion with T-cell predominance lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B18)

This neoplasm is not reportable, nor has an ICD-O-3 code.

The lymph nodes and spleen are the main sites of involvement; less commonly, extranodal sites, including the bone marrow are involved.

Clinical features include lymphadenopathy, hepatosplenomegaly, autoimmune cytopenias, an increased risk of lymphoma, and a high risk of sepsis after splenectomy.

ALPS is associated with an increased risk of B-cell lymphomas, including classic Hodgkin lymphoma, nodular lymphocyte-predominant Hodgkin lymphoma, diffuse large B-cell lymphoma, Burkitt lymphoma, follicular lymphoma, and (rarely) T-cell lymphoma and histiocytic sarcoma.

Alternate Names

ALPS with germline FAS mutation (ALPS-FAS)
ALPS with other specified FAS-pathway germline mutation (FASLG, CASP10, CASP8, FADD)
ALPS with somatic FAS mutation (ALPS-sFAS)
ALPS with unknown underlying mutation

Definition

Autoimmune lymphoproliferative syndrome (ALPS) is an inborn or acquired error of immunity characterized by lymphadenopathy, hepatosplenomegaly, autoimmune cytopenias, and an increased risk of lymphoma. It is associated with germline or somatic defects in the FAS-mediated apoptosis pathway and the accumulation of αβ CD4/CD8 double-negative T (DNT) cells. (WHO 5th edition)

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Tumor-like lesions with T-cell predominance
Pages: Part B: 646-648
Glossary