Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UNIPROT:P04637 (
p53
)
77,613
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The deletion or mutation of the
p53
tumour suppressor gene on chromosome 17p13 is known to be associated with aggressive disease in several B-cell malignancies. The present study describes the
p53
gene status in 20 cases of hairy cell leukemia (HCL) and in 12 cases of its morphological variant (
HCL-V
) by fluorescence in situ hybridization (FISH). A high incidence of
p53
deletion was found in both diseases (75-100% of cases). However, a significant difference was observed between the proportion of cells with
p53
deletion in
HCL-V
cases (mean 31%) and HCL cases (mean 12%) P value < 0.01. The observed difference correlates with the well known tendency for transformation and poor response to therapy in
HCL-V
and seven cases of
HCL-V
with greater than 22% of cells with
p53
deletion showed features of disease progression and transformation. Trisomy 12 was present in 8.5% of the cells in one case of
HCL-V
and in 6-8% of cells in three cases of HCL.
...
PMID:p53 gene deletion and trisomy 12 in hairy cell leukemia and its variant. 1057 9
Hairy cell leukemia variant
(HCLv) is a chronic lymphoproliferative disorder classified as a provisional entity in the 2016 WHO Classification of Lymphoid Tumors. HCLv is characterized by unfavorable prognosis, low complete remission rates and limited disease control following classical hairy cell leukemia-based regimens. In this study, we report 3 cases of elderly patients with treatment-naive,
TP53
un-mutated HCLv, who were effectively treated with four cycles of bendamustine plus rituximab. The regimen was completed in all the patients with acceptable toxicity. All patients achieved a complete clinical response with no evidence of residual disease at bone marrow biopsy and flow-cytometry examination. After a median follow-up of 19 months, the 3 subjects are still in complete remission. In this work, bendamustine plus rituximab proved to be an effective and feasible first-line treatment strategy for elderly patients with
TP53
un-mutated HCLv.
...
PMID:Bendamustine plus rituximab is an effective first-line treatment in hairy cell leukemia variant: a report of three cases. 2929 82
