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Chapter-12 Lymphoproliferative Disorders Associated with Immunodeficiencies and Post-transplantations

BOOK TITLE: Hematopathology Pearls

Author
1. Zhang Da
ISBN
9789352703234
DOI
10.5005/jp/books/14222_13
Edition
2/e
Publishing Year
2018
Pages
4
Author Affiliations
1. The University of Kansas School of Medicine, Kansas City, Kansas, USA, University of Kansas Medical Center, Kansas City, Kansas, USA, University of Kansas Medical Center, Kansas City, Kansas 66160, USA, University of Kansas Medical Center Kansas City, Kansas 66160, USA
Chapter keywords
Lymphoproliferative disorder, immunodeficiency, post-transplantation, Wiskott-Aldrich syndrome, Common variable immunodeficiency disorder, ataxia telangiectasia, CVID, WASP, post-transplant lymphoproliferative disorder, PTLD

Abstract

This chapter describes lymphoproliferative disorders associated with immunodeficiencies and post-transplantations. This chapter covers the Wiskott-Aldrich syndrome, Common variable immunodeficiency disorder, ataxia telangiectasia, and x-linked lymphoproliferative syndrome. Wiskott-Aldrich syndrome is a rare X-linked disorder characterized by thrombocytopenia, small platelets, eczema, recurrent infections, immunodeficiency, and a high incidence of autoimmune diseases and malignancies. Wiskott-Aldrich syndrome protein (WASP) is the key regulator of hematopoietic cells and is involved in cell-signaling, locomotion, and immunologic synapse formation. Common variable immunodeficiency disorder (CVID) is a heterogeneous immunodeficiency disorder clinically characterized by an increased incidence of recurrent infections, autoimmune phenomena and neoplasms.

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