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Blood, 19 November 2009, Vol. 114, No. 21, pp. 4687-4695. Prepublished online as a Blood First Edition Paper on September 10, 2009; DOI 10.1182/blood-2009-01-201731. This Article Full Text (PDF) All Versions of this Article: blood-2009-01-201731v1 114/21/4687    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Google Scholar Articles by Arons, E. Articles by Kreitman, R. J. PubMed PubMed Citation Articles by Arons, E. Articles by Kreitman, R. J. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted January 27, 2009 Accepted August 19, 2009 VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy Evgeny Arons, Tara Suntum, Maryalice Stetler-Stevenson, and Robert J. Kreitman* Laboratory of Molecular Biology, National Cancer Institute, National Institutes of Health, Bethesda, MD, United States Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, United States * Corresponding author; email: kreitmar{at}mail.nih.gov. Hairy cell leukemia variant (HCLv) presents with high disease burden, lack of typical antigens like CD25, and poor response to standard treatments like cladribine. Occasionally, patients with classic HCL respond poorly. Comparison of clinical and molecular features of HCL and HCLv has not been reported. Rearrangements expressing immunoglobulin variable heavy chain were sequenced, including 22 from 20 patients with HCLv and 63 from 62 with classic HCL. Most were seeking relapsed/refractory trials, representing a poor-prognosis population. VH4-34, a gene commonly used in autoimmune disorders, was observed in 8 (40%) HCLv and 6 (10%) classic (p=0.004) HCL patients. Compared to 71 VH4-34-negative rearrangements, 14 VH4-34+ rearrangements were more frequently (p=4x10-8) un-mutated, defined as >98% homologous to germline sequence. VH4-34+ patients had higher white blood cell counts at diagnosis (p=0.002), lower response rate (p=0.00001) and progression-free survival (p=0.007) after initial cladribine, and shorter overall survival from diagnosis (p<0.0001). Response and survival were more closely related to VH4-34 status than to whether or not patients had HCLv. VH4-34+ hairy cell leukemia is an important disorder which only partly overlaps with the previously described HCLv. Response to initial single-agent cladribine therapy is suboptimal; these patients should be considered for alternative approaches including antibody-related therapy. This trial was registered at www.clinicaltrials.gov as nos. NCT00337311, NCT00462189, NCT00074048, and NCT00021983. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: IG genes and hairy cell leukemia Federico Caligaris-Cappio Blood 2009 114: 4610-4611. [Full Text] [PDF] This article has been cited by other articles: F. Caligaris-Cappio IG genes and hairy cell leukemia Blood, November 19, 2009; 114(21): 4610 - 4611. [Full Text] [PDF]

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