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J Allergy Clin Immunol. 2019 Feb 20. pii: S0091-6749(19)30271-4. doi: 10.1016/j.jaci.2019.01.046. [Epub ahead of print]

Lymphoproliferative Disease in Wiskott-Aldrich Syndrome. Analysis of the French Registry of Primary Immunodeficiencies.

Author information

1
Clinical Hematology, Necker University Hospital, AP-HP, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker University Hospital, AP-HP, Paris, France. Electronic address: felipe.suarez@aphp.fr.
2
Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker University Hospital, AP-HP, Paris, France; Pediatric Immuno-Hematology and Rheumatology Unit, Necker University Hospital, AP-HP, Paris, France; INSERM UMR 1163, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Paris, France.
3
Biological Hematology, Kremlin Bicêtre University Hospital, AP-HP, Paris, France.
4
Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; Pathology Department, Necker University Hospital, Paris, AP-HP, Paris, France.
5
Clinical Hematology, Institut Universitaire du Cancer Toulouse-Oncopôle, Toulouse, France.
6
Center for Pathophysiology of Toulouse Purpan, INSERM UMR1043, CNRS UMR5282, Paul Sabatier University, Toulouse, France.
7
Center for Pathophysiology of Toulouse Purpan, INSERM UMR1043, CNRS UMR5282, Paul Sabatier University, Toulouse, France; Fernandes Figueira Institute, Fiocruz, Rio de Janeiro, Brazil.
8
Immunology Department, Saint-Louis University Hospital, AP-HP, Paris, France.
9
Nice University Hospital, University of Côte d'Azur, Nice, France.
10
Clinical Hematology, Pontchaillou University Hospital, Rennes, France.
11
Department of Immuno-Hemato-Pediatrics, Claude Bernard University, IHOP, Lyon, France.
12
Paris Descartes University, Sorbonne Paris Cité, Imagine Institute, Paris, France; French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker University Hospital, AP-HP, Paris, France; Pediatric Immuno-Hematology and Rheumatology Unit, Necker University Hospital, AP-HP, Paris, France; INSERM UMR 1163, Laboratory of Human Genetics of Infectious Diseases, Necker Branch, Paris, France; Collège de France, Paris, France.

Abstract

Aggressive mature B-cell LPD can occur in WAS as in XLT pediatric and adult patients. Immunochemotherapy is applicable to all WAS/XLT patients, while transplantation still remains controversial for adult patients.

KEYWORDS:

Wiskott-Aldrich syndrome; X-linked thrombocytopenia; lymphoma; lymphoproliferative disease; primary immune deficiency

PMID:
30796981
DOI:
10.1016/j.jaci.2019.01.046

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