| Pentostatin for treatment of refractory autoimmune lymphoproliferative syndrome. | |
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MedLine Citation:
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PMID: 21495163 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Autoimmune lymphoproliferative syndrome (ALPS), a disorder of programmed cell death, could be due to a congenital defect in the Fas signaling pathway or other pathways for apoptosis. Most cases present with lymphoproliferation and certain autoimmune features such as thrombocytopenia, neutropenia, and anemia are due to excessive production of antibodies by B lymphocytes. Majority of cases present within the first few years of life. We report a case of ALPS presenting at birth which was refractory to splenectomy and immunosuppressive therapy, but responded to pentostatin followed by hematopoietic stem cell transplantation (HSCT). |
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Authors:
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Rajinder Bajwa; Stephanie Savelli; Thomas Gross |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2011-04-14 |
Journal Detail:
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Title: Pediatric blood & cancer Volume: 57 ISSN: 1545-5017 ISO Abbreviation: Pediatr Blood Cancer Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-06-14 Completed Date: 2011-08-23 Revised Date: 2012-01-23 |
Medline Journal Info:
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Nlm Unique ID: 101186624 Medline TA: Pediatr Blood Cancer Country: United States |
Other Details:
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Languages: eng Pagination: 336-7 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Wiley-Liss, Inc. |
Affiliation:
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Department of Hematology/Oncology/BMT, Nationwide Children's Hospital, Columbus, Ohio 43205, USA. rajinder.bajwa@NationwideChildrens.org |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Antineoplastic Agents
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therapeutic use* Autoimmune Lymphoproliferative Syndrome / drug therapy* Combined Modality Therapy Diseases in Twins / drug therapy* Hematopoietic Stem Cell Transplantation Humans Infant, Newborn Male Pentostatin / therapeutic use* |
| Chemical | |
Reg. No./Substance:
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0/Antineoplastic Agents; 53910-25-1/Pentostatin |
| Comments/Corrections | |
Comment In:
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Pediatr Blood Cancer. 2012 Feb;58(2):310; author reply 311
[PMID:
21674759
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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