NDLI: Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop

Content Provider	PubMed Central
Author	Oliveira, Joao B. Bleesing, Jack J. Umberto, Dianzani Fleisher, Thomas A. Jaffe, Elaine S. Lenardo, Michael J. Frederic, Rieux-laucat Siegel, Richard M. Su, Helen C. Teachey, David T. Rao, V. Koneti
Abstract	Lymphadenopathy in children for which no infectious or malignant cause can be ascertained constitutes a challenging diagnostic dilemma. Autoimmune lymphoproliferative syndrome (ALPS) is a human genetic disorder of lymphocyte apoptosis resulting in an accumulation of lymphocytes and childhood onset chronic lymphadenopathy, splenomegaly, multilineage cytopenias, and an increased risk of B-cell lymphoma. In 1999, investigators at the National Institutes of Health (NIH) suggested criteria to establish the diagnosis of ALPS. Since then, with approximately 500 patients with ALPS studied worldwide, significant advances in our understanding of the disease have prompted the need for revisions to the existing diagnostic criteria and classification scheme. The rationale and recommendations outlined here stem from an international workshop held at NIH on September 21 and 22, 2009, attended by investigators from the United States, Europe, and Australia engaged in clinical and basic science research on ALPS and related disorders. It is hoped that harmonizing the diagnosis and classification of ALPS will foster collaborative research and better understanding of the pathogenesis of autoimmune cytopenias and B-cell lymphomas.
Related Links	http://dx.doi.org/10.1182/blood-2010-04-280347
Starting Page	35
File Format	PDF
ISSN	00064971
e-ISSN	15280020
Journal	Blood
Issue Number	14
Volume Number	116
Language	English
Publisher	American Society of Hematology
Publisher Date	2010-10-07
Access Restriction	Open
Rights Holder	American Society of Hematology
Subject Keyword	Immunology Cell Biology Biochemistry Hematology Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Cell Biology Hematology Biochemistry Immunology

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