Autoimmune Lymphoproliferative Syndrome (ALPS): A Case with Congenital Onset-Reference-Cited by-同舟云学术

Autoimmune Lymphoproliferative Syndrome (ALPS): A Case with Congenital Onset

Published:2007-08 Issue:4 Volume:10 Page:315-319
ISSN:1093-5266
Container-title:Pediatric and Developmental Pathology
language:en
Short-container-title:Pediatr Dev Pathol

Author:

Kahwash Samir B.¹,Fung Bonita¹,Savelli Stephanie²³,Bleesing Jack J.⁴,Qualman Stephen J.¹

Affiliation:

1. Department of Laboratory Medicine, Columbus Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA

2. Department of Pediatrics, Section of Hematology/Oncology, Columbus Children's Hospital, 700 Children's Drive, Columbus, OH 43205, USA

3. Dr. Savelli is currently on staff at Children's Hospital Medical Center of Akron, OH

4. Department of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA

Abstract

We describe a case of autoimmune lymphoproliferative syndrome (ALPS), which is very unusual with regard to a clinical onset soon after birth, and a clinical picture dominated by splenomegaly, jaundice, and consumptive peripheral blood cytopenias, with minimal lymphadenopathy. Our documented close follow up demonstrated initial involvement of the spleen, followed by involvement of the bone marrow and the peripheral blood. The patient underwent bone marrow transplant and is alive and well 20 months after diagnosis.

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology, and Child Health

Link

http://journals.sagepub.com/doi/pdf/10.2350/06-06-0105.1

Reference11 articles.

1. Autoimmune Lymphoproliferative Syndrome (ALPS)

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5. Immunophenotypic profiles in families with autoimmune lymphoproliferative syndrome

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