Linked Life Data

15621772

Source:http://linkedlifedata.com/resource/pubmed/id/15621772

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
12
pubmed:dateCreated
2004-12-28
pubmed:abstractText
Job's or hyper immunoglobulin E recurrent infection syndrome (Hyper-IgE syndrome) is a rare, often inherited multisystem disorder, characterized by cutaneous abscesses, pneumonia, elevated IgE levels and skeletal defects. We report a case of a 22-year-old man with Job's syndrome who presented with back pain. He was found to have diffuse large B-cell lymphoma involving his second lumbar vertebrae and spleen. Treatment with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) chemotherapy achieved a complete remission after 4 cycles. A review of reported cases of lymphoma in Job's syndrome indicates an increase in relative risk of 259 (95% confidence interval 102, 416). The cause of the increased risk has yet to be defined but has similarities to a pathogenetic model of AIDS related lymphoma. In previous reports of lymphoma in Job's syndrome, patients presented with extranodal disease and had poor outcomes. With appropriate chemotherapy and hematological support, lymphoma associated with Job's syndrome can achieve complete remission.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Dec
pubmed:issn
1042-8194
pubmed:author
pubmed:issnType
Print
pubmed:volume
45
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
2521-5
pubmed:dateRevised
2005-11-16
pubmed:meshHeading
pubmed:year
2004
pubmed:articleTitle
Non-Hodgkin's lymphoma in Job's syndrome: a case report and literature review.
pubmed:affiliation
National Cancer Institute, Bethesda, Maryland 20892, USA.
pubmed:publicationType
Journal Article, Review, Case Reports