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

Eszter Sári, Zsolt György Nagy, Kornélia Baghy, Hajnalka Rajnai, Csaba Bödör, Judit Csomor, G. Barna, Gábor Rudas, I. Kovalszky, J. Demeter

Research output: Article

4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)973-980
Number of pages8
JournalPathology and Oncology Research
Volume20
Issue number4
DOIs
Publication statusPublished - okt. 1 2014

Fingerprint

Hairy Cell Leukemia
Lymphoma
Differential Diagnosis
Therapeutics
Cladribine
Purine Nucleosides
Mutation
Inborn Genetic Diseases
Lymphoproliferative Disorders
Lymphocytes
Infection

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Treatment of refractory hairy cell leukemia with a BRAF-inhibitor : lessons to be learnt. / Sári, Eszter; Nagy, Zsolt György; Baghy, Kornélia; Rajnai, Hajnalka; Bödör, Csaba; Csomor, Judit; Barna, G.; Rudas, Gábor; Kovalszky, I.; Demeter, J.

In: Pathology and Oncology Research, Vol. 20, No. 4, 01.10.2014, p. 973-980.

Research output: Article

Sári, Eszter ; Nagy, Zsolt György ; Baghy, Kornélia ; Rajnai, Hajnalka ; Bödör, Csaba ; Csomor, Judit ; Barna, G. ; Rudas, Gábor ; Kovalszky, I. ; Demeter, J. / Treatment of refractory hairy cell leukemia with a BRAF-inhibitor : lessons to be learnt. In: Pathology and Oncology Research. 2014 ; Vol. 20, No. 4. pp. 973-980.
@article{6460dc85e5fd4ccaa8fa23def1dcfc63,
title = "Treatment of refractory hairy cell leukemia with a BRAF-inhibitor: lessons to be learnt",
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.",
author = "Eszter S{\'a}ri and Nagy, {Zsolt Gy{\"o}rgy} and Korn{\'e}lia Baghy and Hajnalka Rajnai and Csaba B{\"o}d{\"o}r and Judit Csomor and G. Barna and G{\'a}bor Rudas and I. Kovalszky and J. Demeter",
year = "2014",
month = "10",
day = "1",
doi = "10.1007/s12253-014-9783-9",
language = "English",
volume = "20",
pages = "973--980",
journal = "Pathology and Oncology Research",
issn = "1219-4956",
publisher = "Springer Netherlands",
number = "4",

}

TY - JOUR

T1 - Treatment of refractory hairy cell leukemia with a BRAF-inhibitor

T2 - lessons to be learnt

AU - Sári, Eszter

AU - Nagy, Zsolt György

AU - Baghy, Kornélia

AU - Rajnai, Hajnalka

AU - Bödör, Csaba

AU - Csomor, Judit

AU - Barna, G.

AU - Rudas, Gábor

AU - Kovalszky, I.

AU - Demeter, J.

PY - 2014/10/1

Y1 - 2014/10/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=84936078643&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84936078643&partnerID=8YFLogxK

U2 - 10.1007/s12253-014-9783-9

DO - 10.1007/s12253-014-9783-9

M3 - Article

C2 - 24789721

AN - SCOPUS:84909609275

VL - 20

SP - 973

EP - 980

JO - Pathology and Oncology Research

JF - Pathology and Oncology Research

SN - 1219-4956

IS - 4

ER -