Biology and Treatment of Hairy Cell Leukemia.
Opinion Statement: Despite its rarity, hairy cell leukemia (HCL) remains a fascinating disease and the physiopathology is becoming more and more understood. The accurate diagnosis of HCL relies on the recognition of hairy cells by morphology and flow cytometry (FCM) in the blood and/or bone marrow (...
| Publicado en: | Current Treatment Options in Oncology Vol. 21; no. 6; pp. 1 - 15 |
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| Autores principales: | , |
| Formato: | review Journal Article |
| Publicado: |
Springer Nature
Jun2020
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=143759530&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 143759530 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 15272729 3KHC jtl: Current Treatment Options in Oncology issn: 15272729 maglogo: N pubinfo: dt: Jun2020 vid: 21 iid: 6 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 143759530 143759530 NLM32350628 143759530 10.1007/s11864-020-00732-0 NLM32350628 143759530 ppf: 1 ppct: 14 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Biology and Treatment of Hairy Cell Leukemia. aug: au: Paillassa, Jérôme Troussard, Xavier affil: Department of Hematology, Academic Hospital of Angers, Angers, Pays de la Loire, France sug: subj: Leukemia Therapy Leukemia Etiology Immunophenotyping Disease Management Signal Transduction Animals Bone Marrow Pathology Outcomes (Health Care) Leukemia Diagnosis Disease Progression Combined Modality Therapy Methods Treatment Outcomes Repeat Procedures Disease Susceptibility Histocytochemistry Biopsy Leukemia Mortality Mutation Scales ab: Opinion Statement: Despite its rarity, hairy cell leukemia (HCL) remains a fascinating disease and the physiopathology is becoming more and more understood. The accurate diagnosis of HCL relies on the recognition of hairy cells by morphology and flow cytometry (FCM) in the blood and/or bone marrow (BM). The BRAF V600E mutation, an HCL-defining mutation, represents a novel diagnostic parameter and a potential therapeutic target. The precise cellular origin of HCL is a late-activated postgerminal center memory B cell. BRAF mutations were detected in hematopoietic stem cells (HSCs) of patients with HCL, suggesting that this is an early HCL-defining event. Watch-and-wait strategy is necessary in approximately 10% of asymptomatic HCL patients, sometimes for several years. Purine analogs (PNAs) are the established first-line options for symptomatic HCL patients. In second-line treatment, chemoimmunotherapy combining PNA plus rituximab should be considered in high-risk HCL patients. The three options for relapsed/refractory HCL patients include recombinant immunoconjugates targeting CD22, BRAF inhibitors, and BCR inhibitors. The clinical interest to investigate blood minimal residual disease (MRD) was recently demonstrated, with a high risk of relapse in patients with positive testing for MRD and a low risk in patients with negative testing. However, efforts must be made to standardize MRD analyses in the near future. Patients with HCL are at risk of second malignancies. The increased risk could be related to the disease and/or the treatment, and the respective role of PNAs in the development of secondary malignancies remains a topic of debate. pubtype: Academic Journal doctype: review Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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