Document Detail


[Recurrent somatic mutation in hairy cell leukemia].
MedLine Citation:
PMID:  23335721     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Hairy cell leukemia is a mature B-cell non-Hogkin lymphoma characterized by unique clinical, morphological and immunhistochemical features. Patients with hairy cell leukemia usually present with splenomegaly, progressive pancytopenia and a relative indolent clinical course. The diagnosis does not always indicate immediate treatment, as treatment depends on the clinical stage of the leukemia. Asymptomatic disease without progression requires a watchful waiting policy, while other categories usually need treatment. The treatment of choice is purin nucleosid analogues (pentostatin, cladribine) which can achieve complete remission even for decades. Interferon and monoclonal CD20 antibodies can also significantly prolong tevent free survival. Unfortunately, only the latter two therapies are easily available in Hungary. Splenectomy, which was suggested as first line treatment before the era of purin nucleosid analogues, is only recommended as ultimum refugium. Although hairy cell leukemia is a well-defined lymphoproliferative disease, sometimes it is difficult to differentiate it from other similar entities such as hairy cell leukema variant, splenic marginal zone lymphoma, small lymphocytic lymphoma etc. Making the correct diagnosis is of utmost importance because of the great difference in treatment modalities. Recently, a somatic mutation was found in all analysed hairy cell leukemia samples, but not in other splenic B-cell lymphomas. This article reviews the significance of this observation and presents the different types of methods for the detection of this mutation. Orv. Hetil., 2013, 154, 123-127.
Authors:
Eszter Sári; Zsolt Nagy; Judit Demeter
Publication Detail:
Type:  English Abstract; Journal Article    
Journal Detail:
Title:  Orvosi hetilap     Volume:  154     ISSN:  0030-6002     ISO Abbreviation:  Orv Hetil     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-01-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376412     Medline TA:  Orv Hetil     Country:  Hungary    
Other Details:
Languages:  hun     Pagination:  123-7     Citation Subset:  IM    
Affiliation:
Semmelweis Egyetem, Általános Orvostudományi Kar I. Belgyógyászati Klinika Budapest Korányi S. u. 2/a 1083.
Vernacular Title:
Visszatérő szomatikus mutáció hajas sejtes leukémiában.
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