Hairy Cell Leukemia
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Associated Genetic Biomarkers
NCI Definition: A neoplasm of small B-lymphocytes with "hairy" projections in bone marrow, spleen, and peripheral blood. Most patients present with splenomegaly and pancytopenia. (WHO, 2001) 
Hairy cell leukemias most frequently harbor alterations in BRAF, ATM, NF1, EP400, and DNMT3A .
BRAF V600E, BRAF Mutation, BRAF Exon 15 Mutation, BRAF Codon 600 Missense, and ATM Mutation are the most common alterations in hairy cell leukemia .
Of the biomarker-directed therapies for hairy cell leukemia, 0 are FDA-approved in at least one setting and 1 has NCCN guidelines in at least one setting .
Disease is predicted to be sensitive: -
Sample must match all of the following:
|Clinical Setting(s): Refractory (NCCN), Relapse (NCCN)|
|Note: Hairy Cell Leukemia, a subtype of Mature B-Cell Lymphoma/Leukemia, harboring BRAF V600E mutation responded to vemurafenib in two patients who had refractory disease or were in relapse after conventional therapies.|
There are 19 clinical trials for hairy cell leukemia, of which 17 are open and 2 are completed or closed. Of the trials that contain hairy cell leukemia as an inclusion criterion, 5 are phase 1 (5 open), 3 are phase 1/phase 2 (1 open), and 11 are phase 2 (11 open).
BRAF, CD22, and MS4A1 are the most frequent gene inclusion criteria for hairy cell leukemia clinical trials .
Rituximab, obinutuzumab, and bendamustine are the most common interventions in hairy cell leukemia clinical trials.
Significant Genes in Hairy Cell Leukemia
BRAF is altered in 71.05% of hairy cell leukemia patients .
BRAF is an inclusion eligibility criterion in 4 clinical trials for hairy cell leukemia, of which 4 are open and 0 are closed. Of the trials that contain BRAF status and hairy cell leukemia as inclusion criteria, 4 are phase 2 (4 open) .
Vemurafenib has evidence of efficacy in patients with BRAF mutation in hairy cell leukemia .
MYC is an inclusion eligibility criterion in 1 clinical trial for hairy cell leukemia, of which 1 is open and 0 are closed. Of the trial that contains MYC status and hairy cell leukemia as inclusion criteria, 1 is phase 1/phase 2 (1 open) .
1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.
3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.