Linked Life Data

15339696

Source:http://linkedlifedata.com/resource/pubmed/id/15339696

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
8
pubmed:dateCreated
2004-9-1
pubmed:abstractText
Nijmegen breakage syndrome (NBS) is a rare autosomal-recessive disorder characterized by microcephaly, immunodeficiency and predisposition to cancer, mainly B-cell lymphomas. Our 10-years-old female patient with NBS developed T-cell lymphoblastic leukemia/lymphoma (TLBL/ALL). The use of standard chemotherapy in our patient, except for cranial irradiation, led to complete and sustained remission of TLBL/ALL. In patients with chromosomal instability syndromes chemotherapy must be modified and radiotherapy must be omitted because of potentially serious toxic complications. Careful prevention of infections, including the use of intravenous immunoglobulin is also essential for successful treatment of lymphoid malignancies in NBS. Immunodeficiency in NBS is profound affecting both humoral and cellular immune system. During long-term follow-up after treatment of malignancy our patient remained free of major infections However, the rise of oligoclonal serum IgM was detected recently. Monitoring of serum IgM concentration may be a useful indicator for early detection of lymphomas in NBS.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
1592-8721
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
89
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
ECR27
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
2004
pubmed:articleTitle
T-cell lymphoblastic leukemia/lymphoma in Nijmegen breakage syndrome.
pubmed:affiliation
Department of Pediatric Immunology, Mother and Child Health Institute, Belgrade, Serbia and Montenegro. bg.pasic@eunet.yu
pubmed:publicationType
Journal Article, Case Reports