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Tytuł pozycji:

Hairy cell leukemia: Update on molecular profiling and therapeutic advances.

Tytuł:
Hairy cell leukemia: Update on molecular profiling and therapeutic advances.
Autorzy:
Grever MR; 395 W. 12th Ave, Room 392, Columbus, OH 43210, USA; Department of Internal Medicine, Division of Hematology at The Ohio State University, Columbus, OH, USA. Electronic address: .
Blachly JS; 320 W. 10th Ave, 406C Starling Loving Hall, Columbus, OH 43210, USA; Department of Internal Medicine, Division of Hematology at The Ohio State University, Columbus, OH, USA. Electronic address: .
Andritsos LA; 320 W. 10th Ave, A352 Starling Loving Hall, Columbus, OH 43210, USA; Department of Internal Medicine, Division of Hematology at The Ohio State University, Columbus, OH, USA. Electronic address: .
Źródło:
Blood reviews [Blood Rev] 2014 Sep; Vol. 28 (5), pp. 197-203. Date of Electronic Publication: 2014 Jul 11.
Typ publikacji:
Journal Article; Review
Język:
English
Imprint Name(s):
Publication: 2001- : London : Elsevier
Original Publication: [Edinburgh ; New York] : Churchill Livingstone, [c1987-
MeSH Terms:
Leukemia, Hairy Cell/*diagnosis
Leukemia, Hairy Cell/*therapy
Biomedical Research ; Disease Management ; Humans ; Immunophenotyping ; Infections/etiology ; Infections/therapy ; Leukemia, Hairy Cell/complications ; Leukemia, Hairy Cell/etiology ; Molecular Diagnostic Techniques
Contributed Indexing:
Keywords: Hairy cell leukemia
Entry Date(s):
Date Created: 20140812 Date Completed: 20150522 Latest Revision: 20191210
Update Code:
20240104
DOI:
10.1016/j.blre.2014.06.003
PMID:
25110197
Czasopismo naukowe
Hairy cell leukemia was initially described as a clinicopathologic entity more than 50 years ago. We have subsequently discovered that HCL is really at least two diseases: classical HCL and the hairy cell leukemia variant. The former is among a small group of cancers exceptional for being (nearly) unified by a single genetic lesion, the BRAF V600E mutation. Over the past three decades, tremendous progress in both diagnostic and prognostic clarification has been accompanied by therapeutic advances in classical HCL. Consequently, this once uniformly fatal disease has been converted in most cases into a chronic illness enabling patients to live long and productive lives. In response to standard therapy, patients have high complete remission rates. Unfortunately, the long-term survival curves have not plateaued, revealing that this disease is controlled but not cured. Though rare and representing only about 10% of an already rare disease, those patients with the variant fare exceptionally poorly with standard therapy: complete response rates to purine nucleoside analogs are reported to be less than 50%, whereas the complete response rates in classical HCL are up to 90%. Novel small molecules targeting BRAF and the B-cell receptor signaling complex, and biologic agents like antibodies and immunotoxin conjugates are being explored for those patients who have relapsed. Substantial opportunities for continued research remain. This complex and multi-faceted disease incorporates challenges from altered immunity associated with the underlying disease and its treatments. Considering the rarity of this malignancy, optimization of patient management requires multi-institutional collaboration. The Hairy Cell Leukemia Foundation (www.hairycellleukemia.org) was formed to coordinate these efforts.
(Copyright © 2014. Published by Elsevier Ltd.)

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