[Inherited lymphoproliferative disorders].

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Lymphoproliferative disorders (LPDs) are caused by dysregulated lymphocyte proliferation and include polyclonal benign and monoclonal malignant diseases. LPDs frequently occur in immunocompromized patients, particularly those with primary immunodeficiency disease (PID), a monogenic disease. PID-associated LPD corresponds to inherited LPD. Here, we describe inherited LPD and focus on IKZF1-associated diseases and Epstein-Barr virus-associated LPD, such as ZAP70 deficiency and X-linked lymphoproliferative syndrome type 1 with somatic reversion mosaicism. Disclosing the pathogenesis of inherited LPDs would lead to a broad understanding of LPDs and development of new treatment strategies.

Related collections

Author and article information

Journal

Journal ID (iso-abbrev): Rinsho Ketsueki

Title: [Rinsho ketsueki] The Japanese journal of clinical hematology

Publisher:

ISSN: 0485-1439

ISSN (Print): 0485-1439

Publication date (Electronic): 2019

Volume: 60

Issue: 6

Affiliations

[1 ] Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University.

[2 ] Laboratory of Lymphocyte Activation and Susceptibility to EBV infection, Institut National de la Sante et de la Recherche Medicale UMR 1163.

Article

DOI: 10.11406/rinketsu.60.708

PubMed ID: 31281164

SO-VID: ff97a286-5fa1-4426-b10c-74b21243c047

History

Keywords: Lymphoproliferative disorder,X-linked lymphoproliferative syndrome,ZAP70,IKZF1

Data availability:

Keywords: Lymphoproliferative disorder, X-linked lymphoproliferative syndrome, ZAP70, IKZF1

Comments

Comment on this article

Cited by 2

See all cited by