Journal
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
Volume 97, Issue 6, Pages 926-930Publisher
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.2010.036798
Keywords
A20; ABIN; ocular adnexal MALT lymphoma
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Funding
- Leukaemia & Lymphoma Research, UK
- Elimination of Leukaemia Fund, UK
- Lady Tata Memorial Trust
- Kay Kendal Leukaemia Fund, UK
- Pathological Society of Great Britain and Ireland
- MRC [MC_U117584209] Funding Source: UKRI
- Medical Research Council [MC_U117584209] Funding Source: researchfish
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Recent studies showed A20 inactivation by deletion, mutation and promoter methylation in ocular adnexal mucosa-associated lymphoid tissue lymphoma. However, the incidences of A20 abnormalities and their clinical impact remain for the most part unknown. It is also unknown whether ABIN-1 and ABIN-2, the components of the A20 NF-kappa B inhibitor complex, are inactivated by genetic changes in ocular adnexal mucosa-associated lymphoid tissue lymphoma. A total of 105 cases were investigated for A20 mutation/deletion, ABIN-1/2 mutation, MALT1 and IGH involved translocation. Somatic mutation was seen frequently in A20 (28.6%) but rarely in ABIN-1 (1%) and ABIN-2 (1%). A20 mutations were significantly associated with A20 heterozygous deletion, and both were mutually exclusive from the MALT1 or IGH involved translocations. A20 mutation/deletion was also significantly associated with increased expression of the NF-kappa B target genes CCR2, TLR6 and BCL2. The cases with A20 mutation/deletion required significantly higher radiation dosages to achieve complete remission than those without these abnormalities.
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