|
Hairy cell leukemia variant
|
0.300 |
SusceptibilityMutation
|
disease |
ORPHANET |
Molecular variant of hairy cell leukemia with poor prognosis.
|
21599610 |
2011 |
|
Hairy cell leukemia variant
|
0.300 |
SusceptibilityMutation
|
disease |
ORPHANET |
VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy.
|
19745070 |
2009 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
GeneticVariation
|
disease |
BEFREE |
Chronic Lymphocytic Leukemia with Mutated IGHV4-34 Receptors: Shared and Distinct Immunogenetic Features and Clinical Outcomes.
|
28536306 |
2017 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
GeneticVariation
|
disease |
BEFREE |
Chronic lymphocytic leukemia (CLL) patients assigned to stereotyped subset #4 (mutated IGHV4-34/IGKV2-30 BCR Ig) display a particularly indolent disease course.
|
27059597 |
2016 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
Biomarker
|
disease |
BEFREE |
In this study we investigated specific biological and clinical features associated with chronic lymphocytic leukemia (CLL) patients carrying stereotyped BCR subset #4 (IGHV4-34) among a prospective cohort of 462 CLL/MBL patients in early stage (Binet A).
|
25860243 |
2015 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
Biomarker
|
disease |
BEFREE |
Temporal dynamics of clonal evolution in chronic lymphocytic leukemia with stereotyped IGHV4-34/IGKV2-30 antigen receptors: longitudinal immunogenetic evidence.
|
23922244 |
2013 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
AlteredExpression
|
disease |
BEFREE |
We recently showed that ID in the IG heavy chain genes of patients with chronic lymphocytic leukemia (CLL) was generally limited; however, intense ID was evident in selected cases, especially those expressing stereotyped IGHV4-34 rearrangements and assigned to subset 4.
|
20463750 |
2010 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
AlteredExpression
|
disease |
BEFREE |
Distinct gene expression profiles in subsets of chronic lymphocytic leukemia expressing stereotyped IGHV4-34 B-cell receptors.
|
20801898 |
2010 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
AlteredExpression
|
disease |
BEFREE |
In this context, we sought evidence for persistent activation by EBV and CMV in CLL cases expressing the IGHV4-34 gene.
|
19148139 |
2009 |
|
Chronic Lymphocytic Leukemia
|
0.080 |
GeneticVariation
|
disease |
BEFREE |
Subsets with "CLL-biased" homologous complementarity-determining region 3 (CDR3) were identified: (1) IGKV2-30-IGKJ2, 7 sequences with homologous kappa CDR3 (KCDR3), 5 of 7 associated with homologous IGHV4-34 heavy chains; (2) IGKV1-39/1D-39-IGKJ1/4, 4 unmutated sequences with homologous KCDR3, 2 of 4 associated with homologous IGHV4-39 heavy chains; (3) IGKV1-5-IGKJ1/3, 4 sequences with homologous KCDR3, 2 of 4 associated with unmutated nonhomologous IGHV4-39 heavy chains; (4) IGLV1-44-IGLJ2/3, 2 sequences with homologous lambda CDR3 (LCDR3), associated with homologous IGHV4-b heavy chains; and (5) IGLV3-21-IGLJ2/3, 9 sequences with homologous LCDR3, 3 of 9 associated with homologous IGHV3-21 heavy chains.
|
16076869 |
2005 |
|
Hairy Cell Leukemia
|
0.040 |
GeneticVariation
|
disease |
BEFREE |
A molecularly defined IGHV4-34+ variant is also resistant whether HCL or HCLv immunophenotypically.
|
31068044 |
2019 |
|
Hairy Cell Leukemia
|
0.040 |
GeneticVariation
|
disease |
BEFREE |
HCL variant and the IGHV4-34 molecular variant of HCL lack BRAF mutation and have inferior outcomes with standard purine analogue therapy.
|
28146266 |
2017 |
|
Hairy Cell Leukemia
|
0.040 |
GeneticVariation
|
disease |
BEFREE |
High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias.
|
24241536 |
2014 |
|
Hairy Cell Leukemia
|
0.040 |
Biomarker
|
disease |
BEFREE |
Our results suggest that HCLv and IGHV4-34(+) HCLs have a different pathogenesis than HCLc and that a significant minority of other HCLc are also wild-type for BRAF V600.
|
22210875 |
2012 |
|
Mucosa-Associated Lymphoid Tissue Lymphoma
|
0.020 |
Biomarker
|
disease |
BEFREE |
We showed that: (1) there was a significant association between the biased usage of IGHV4-34 (binds to the carbohydrate I/i antigens) and inactivating mutation of TNFAIP3 [encoding a global negative regulator of the canonical nuclear factor-κB (NF-κB) pathway] in ocular adnexal MALT lymphoma; (2) IGHV1-69 was significantly overrepresented (54%) in MALT lymphoma of the salivary gland, but was not associated with mutation in any of the 17 genes investigated; and (3) MALT lymphoma lacked mutations that are frequently seen in other B-cell lymphomas characterized by constitutive NF-κB activities, including mutations in CD79B, CARD11, MYD88, TNFRSF11A, and TRAF3.
|
28682481 |
2017 |
|
Mucosa-Associated Lymphoid Tissue Lymphoma
|
0.020 |
GeneticVariation
|
disease |
BEFREE |
The list is now growing with the report of increased frequency of inactivating mutations in the TNFAIP3 gene in MALT lymphomas expressing IG receptors encoded by the IGHV4-34 gene, particularly of the ocular adnexa.
|
28892161 |
2017 |
|
Primary central nervous system lymphoma
|
0.020 |
Biomarker
|
disease |
BEFREE |
Furthermore, 87% (20/23) of the recAbs, including all Abs derived from IGHV4-34 using PCNSL, recognized galectin-3, which was upregulated on microglia/macrophages, astrocytes, and cerebral endothelial cells upon CNS invasion by PCNSL.
|
26116512 |
2015 |
|
Lymphoproliferative Disorders
|
0.020 |
GeneticVariation
|
group |
BEFREE |
The absence of plasmacytoid cells, the presence of plasma cells predominantly outside the nodular lymphoid infiltrates, IGHV4-34 restriction and absence of MYD88 L265P mutation strongly suggest that cold agglutinin-associated lymphoproliferative disease is a distinct entity that is different from lymphoplasmacytic lymphoma.
|
24143001 |
2014 |
|
Primary central nervous system lymphoma
|
0.020 |
GeneticVariation
|
disease |
BEFREE |
Immunoglobulin heavy-chain variable gene segment (IGHV), IGHV4, was the predominant family used by 66% (33 of 50) of PCNSLs with a preferential rearrangement of the IGHV4-34 gene segment (18 [55%] of 33).
|
25383641 |
2014 |
|
Lymphoproliferative Disorders
|
0.020 |
AlteredExpression
|
group |
BEFREE |
The IGHV4-34 gene was most frequently expressed in chronic lymphocytic leukemia (27.5%), whereas it was rarely found in leukemic chronic lymphoproliferative disorders (7.7%, P = 0.005).
|
19220298 |
2009 |
|
Burkitt Lymphoma
|
0.010 |
Biomarker
|
disease |
BEFREE |
These include IGHV4-34, known to produce autoreactive antibodies, and IGKV3-20, a feature described in other B-cell malignancies but not yet in BL.
|
30617194 |
2019 |
|
Malignant Neoplasms
|
0.010 |
Biomarker
|
group |
BEFREE |
These include IGHV4-34, known to produce autoreactive antibodies, and IGKV3-20, a feature described in other B-cell malignancies but not yet in BL.
|
30617194 |
2019 |
|
Adult Burkitt Lymphoma
|
0.010 |
Biomarker
|
disease |
BEFREE |
These include IGHV4-34, known to produce autoreactive antibodies, and IGKV3-20, a feature described in other B-cell malignancies but not yet in BL.
|
30617194 |
2019 |
|
Childhood Burkitt Lymphoma
|
0.010 |
Biomarker
|
disease |
BEFREE |
These include IGHV4-34, known to produce autoreactive antibodies, and IGKV3-20, a feature described in other B-cell malignancies but not yet in BL.
|
30617194 |
2019 |
|
Lupus Erythematosus, Systemic
|
0.010 |
Biomarker
|
disease |
BEFREE |
Furthermore, subset 4 IGs do not bind DNA nor i or I carbohydrate antigens, common targets of IGHV4-34-utilizing antibodies in systemic lupus erythematosus and cold agglutinin disease, respectively.
|
28097289 |
2017 |