Visszatéro szomatikus mutáció hajas sejtes leukémiában

Eszter Sári, Zsolt Nagy, J. Demeter

Research output: Article

1 Citation (Scopus)

Abstract

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.

Original languageHungarian
Pages (from-to)123-127
Number of pages5
JournalOrvosi Hetilap
Volume154
Issue number4
DOIs
Publication statusPublished - jan. 1 2013

Fingerprint

Hairy Cell Leukemia
Mutation
Therapeutics
Lymphoma
Pentostatin
Cladribine
Watchful Waiting
Asymptomatic Diseases
Pancytopenia
Hungary
Splenomegaly
B-Cell Lymphoma
Splenectomy
B-Cell Chronic Lymphocytic Leukemia
Interferons
Disease Progression
Leukemia
B-Lymphocytes
Monoclonal Antibodies
Survival

Keywords

  • BRAF
  • hairy cell leukemia

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Visszatéro szomatikus mutáció hajas sejtes leukémiában. / Sári, Eszter; Nagy, Zsolt; Demeter, J.

In: Orvosi Hetilap, Vol. 154, No. 4, 01.01.2013, p. 123-127.

Research output: Article

Sári, Eszter ; Nagy, Zsolt ; Demeter, J. / Visszatéro szomatikus mutáció hajas sejtes leukémiában. In: Orvosi Hetilap. 2013 ; Vol. 154, No. 4. pp. 123-127.
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