NDLI: Treatment of refractory hairy cell leukemia with a BRAF-inhibitor: lessons to be learnt.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Sári, Eszter Nagy, Zsolt György Baghy, Kornélia Rajnai, Hajnalka Bödör, Csaba Csomor, Judit Barna, Gábor Rudas, Gábor Kovalszky, Ilona Demeter, Judit
Description	Country affiliation: Hungary Author Affiliation: Sári E ( 1st Department of Internal Medicine, Semmelweis University, Budapest, Korányi S. Str. 2/a, H-1083, Hungary.)
Abstract	Hairy cell leukemia is a rare chronic lymphoproliferative disorder with indolent but progressive clinical course. Patients require treatment when they have significant cytopenia or recurrent infections. The gold standard treatment are purine nucleoside analogues (cladribine and pentostatine), with these agents the rate of complete remission can approach even 95 %. The differential diagnosis between classical hairy cell leukemia and other, rare splenic lymphomas that can mimic this disease might be really challenging. Splenic lymphoma with villous lymphocytes and other new, provisional WHO entities share some, but not all immunophenotypical features with hairy cell leukemia. The correct diagnosis is of an extreme importance as these entities require different treatment. Thus further investigation in the pathogenesis of hairy cell leukemia is required in order to solve this challenge. Discovery of the BRAF V600E mutation as a disease-defining genetic event in hairy cell leukemia can be helpful in both differential diagnosis and treatment of this disease. We report the case of three hairy cell leukemia patients, whose diagnosis or treatment was based on this newly discovered somatic mutation, but the treatment results and side effects were individual.
File Format	HTM / HTML
ISSN	12194956
Issue Number	4
Volume Number	20
e-ISSN	15322807
Journal	Pathology & Oncology Research
Language	English
Publisher	Springer
Publisher Date	2014-10-01
Publisher Place	Netherlands
Access Restriction	Subscribed
Subject Keyword	Discipline Oncology Antineoplastic Agents Therapeutic Use Drug Resistance, Neoplasm Genetics Leukemia, Hairy Cell Drug Therapy Mutation Proto-oncogene Proteins B-raf Antagonists & Inhibitors Adult Aged Humans Male Middle Aged Treatment Outcome Case Reports Journal Article
Content Type	Text
Resource Type	Case study Article
Subject	Cancer Research Pathology and Forensic Medicine Oncology

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