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[Lymphoproliferative disorders and inborn errors of immunity]

Takehiko Doi1, Satoshi Okada1

  • 1Department of Pediatrics, Hiroshima University Institute of Biomedical and Health Sciences.

[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|November 9, 2020
PubMed

Lymphoproliferative disease (LPD) is a comprehensive concept covering diseases ranging from transient lymphadenopathy to lymphoma. LPD is frequently associated with Epstein-Barr virus (EBV) infections and tends to occur in patients with inborn errors of immunity (IEI) and in patients after organ transplantation. Most patients with severe combined immunodeficiency or X-linked lymphoproliferative disease develop LPD. Autoimmune lymphoproliferative syndrome (ALPS), a typical LPD disease, is caused by germline mutations in FAS, FASL, CASP10, CASP8 and FADD, which are involved in the apoptosis pathway. ALPS patients develop autoimmune diseases and LPDs such as hepatosplenomegaly and lymphadenopathy. On the other hand, RAS-associated ALPS-like syndrome and CTLA4 haploinsufficiency also belong to ALPS-associated diseases. EBV-associated LPD is a clinical condition that should be noted in patients with IEI. Patients with genetic defects in SH2D1A, XIAP, CD27, CD70, CD137, ITK, CTPS, RASGRP1, and MAGT1 are prone to EBV-associated LPD.

Keywords:
Epstein-Barr virusImmunodeficiency-associated lymphoproliferative disordersInborn errors of immunityLymphoma

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