Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 15, Pages -Publisher
MDPI
DOI: 10.3390/ijms22157780
Keywords
hairy cell leukemia; HCL; biology; microenvironment; BRAF V600E; DUSP; single-cell sequencing; vitronectin; fibronectin; JNK; p38; B-cell receptor; epigenetic; methylome; microRNA
Ask authors/readers for more resources
Classic hairy cell leukemia is a rare B-cell malignancy characterized by pancytopenia and infectious complications. The key activating mutation BRAF V600E has been identified, but additional oncogenic biologic features are still under investigation for HCL transformation.
Classic hairy cell leukemia (HCL) is a rare mature B-cell malignancy associated with pancytopenia and infectious complications due to progressive infiltration of the bone marrow and spleen. Despite tremendous therapeutic advances achieved with the implementation of purine analogues such as cladribine into clinical practice, the culprit biologic alterations driving this fascinating hematologic disease have long stayed concealed. Nearly 10 years ago, BRAF V600E was finally identified as a key activating mutation detectable in almost all HCL patients and throughout the entire course of the disease. However, additional oncogenic biologic features seem mandatory to enable HCL transformation, an open issue still under active investigation. This review summarizes the current understanding of key pathogenic mechanisms implicated in HCL and discusses major hurdles to overcome in the context of other BRAF-mutated malignancies.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available