| Pulmonary Lymphomatoid Granulomatosis in Griscelli Syndrome Type 2. | |
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MedLine Citation:
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PMID: 22111599 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Abstract Griscelli syndrome type 2 (GS2) is a rare autosomal-recessive disorder associated with a RAB27A gene mutation, and clinically manifesting as hypopigmentation, disseminated chronic encephalitis, and severe immunological disorders characterized by an accelerated hematological phase, also referred to as hemophagocytic syndrome (HS), or hemophagocytic lymphohistiocytosis (HLH). The authors report the diagnosis of GS2 in an 11-year-old girl with hypopigmentation, immunodeficiency, hepatosplenomegaly, severe neurological impairments, and fatal multiorgan failure. In this patient a diagnosis of pulmonary lymphomatoid granulomatosis (LG), an Epstein-Barr virus (EBV)-related lymphoproliferative disorder, was established from radiological and histological findings. Although EBV-related malignancies are common in immunocompromised patients, this is the first report of a diagnosis of pulmonary LG in a patient with GS2. |
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Authors:
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Aleksandra Szczawinska-Poplonyk; Zdzislawa Kycler; Anna Breborowicz; Maja Klaudel-Dreszler; Malgorzata Pac; Maria Zegadlo-Mylik; Renata Langfort |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-11-23 |
Journal Detail:
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Title: Viral immunology Volume: - ISSN: 1557-8976 ISO Abbreviation: - Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-11-24 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8801552 Medline TA: Viral Immunol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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1 Department of Pediatric Pneumonology, Allergology, and Clinical Immunology, 3rd Chair of Pediatrics, Poznan University of Medical Sciences , Poznan, Poland . |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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