Combined immunodeficiency and Epstein-Barr virus-induced B cell malignancy in humans with inherited CD70 deficiency.

doi:10.1084/jem.20160849

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Article

Combined immunodeficiency and Epstein-Barr virus-induced B cell malignancy in humans with inherited CD70 deficiency.

The Journal of experimental medicine - 1 Jan 2017

Abolhassani Hassan, Edwards Emily S J, Ikinciogullari Aydan, Jing Huie, Borte Stephan, Buggert Marcus, Du Likun, Matsuda-Lennikov Mami, Romano Rosa, Caridha Rozina, Bade Sangeeta, Zhang Yu, Frederiksen Juliet, Fang Mingyan, Bal Sevgi Kostel, Haskologlu Sule, Dogu Figen, Tacyildiz Nurdan, Matthews Helen F, McElwee Joshua J, Gostick Emma, Price David A, Palendira Umaimainthan, Aghamohammadi Asghar, Boisson Bertrand, Rezaei Nima, Karlsson Annika C, Lenardo Michael J, Casanova Jean-Laurent, Hammarström Lennart, Tangye Stuart G, Su Helen C, Pan-Hammarström Qiang

Abstract excerpt

In this study, we describe four patients from two unrelated families of different ethnicities with a primary immunodeficiency, predominantly manifesting as susceptibility to Epstein-Barr virus (EBV)-related diseases. Three patients presented with EBV-associated Hodgkin's lymphoma and hypogammaglobulinemia; one also had severe varicella infection. The fourth had viral encephalitis during infancy. Homozygous...

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PMID: 28011864
DOI: 10.1084/jem.20160849
PMCID: PMC5206499

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