Extranasal extranodal NK/T-cell lymphoma associated with systemic lupus erythematosus

International Journal of Hematology
Satoshi IchikawaHideo Harigae

Abstract

Increased incidence of lymphoproliferative disorders is reported in patients with autoimmune diseases, majority of which have a B-cell phenotype and are pathogenetically associated with the reactivation of Epstein-Barr virus (EBV). However, EBV-associated T/NK-cell lymphoma has hardly been reported. We present the case of a 68-year-old-woman, who had been diagnosed with systemic lupus erythematosus (SLE) 28 years back and was treated with various immunosuppressive agents including steroids, cyclophosphamide, and tacrolimus. She presented with a progressively worsening swelling of the right thigh for the last few months. Radiological examination revealed an intramuscular bulky tumor without any other lesions and the biopsy results led to a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKL). Concurrent chemoradiotherapy resulted in a complete response, which has been sustained for more than 2 years without requiring additional therapy. After the initiation of chemotherapy, SLE did not worsen with the administration of low-dose corticosteroids. To the best of our knowledge, this is the first case report of a localized extranasal ENKL developing in a patient with SLE.

References

Jul 23, 2003·Lupus·Ming-Hsien LinChao-Jung Tsao
Jan 14, 2004·Kidney International·Jan J WeeningUNKNOWN Renal Pathology Society Working Group on the Classification of Lupus Nephritis
Feb 14, 2007·Clinical Rheumatology·Jennifer K King, Karen H Costenbader
Oct 7, 2009·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Motoko YamaguchiKazuo Oshimi
Dec 1, 2010·International Journal of Hematology·Ritsuro Suzuki
May 2, 2012·Annals of Oncology : Official Journal of the European Society for Medical Oncology·H K AhnW S Kim
May 5, 2012·European Journal of Haematology·Jae-Cheol JoCheolwon Suh
Jan 11, 2013·Annals of the Rheumatic Diseases·Sasha BernatskyAnn E Clarke
Feb 16, 2013·Journal of Autoimmunity·Sasha BernatskyAnn E Clarke
Jan 7, 2015·Scandinavian Journal of Rheumatology·N S RasmussenG Houen
Jul 1, 2015·Current Opinion in Rheumatology·Gillian C GoobieAnn E Clarke
Dec 12, 2017·Best Practice & Research. Clinical Rheumatology·May Y ChoiAnn E Clarke
Nov 21, 2018·Leukemia Research·Alina KleinAnat Gafter-Gvili
Feb 27, 2020·International Journal of Molecular Sciences·Atif Saleem, Yasodha Natkunam

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Autoimmune Lymphoproliferative Syndrome

Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. Discover the latest research on ALPS here.

© 2022 Meta ULC. All rights reserved