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Haarzellleukämie

Hairy cell leukemia

  • Leitthema
  • Published:
Der Onkologe Aims and scope

Zusammenfassung

Hintergrund

Die Haarzellleukämie (HZL) gehört zu den indolenten B‑Zell-Lymphomen. Das mediane Alter bei Erstdiagnose liegt bei 45–55 Jahren, Männer sind etwa 4‑mal häufiger als Frauen betroffen. Die klinischen Symptome sind zytopenieassoziiert (Neutropenie, Thrombozytopenie, Anämie), dazu kommen Splenomegalie und Fatigue.

Ergebnisse

Innerhalb der HZL werden die häufige klassische HZL und die seltene HZL-Variante unterschieden. Bei der klassischen HZL werden durch die Therapie mit Purinanaloga Remissionsraten von über 95 % erzielt. Weitere wirksame Behandlungen sind die Immuntherapie und BRAF-Inhibitoren.

Schlussfolgerung

Die HZL ist eine chronische Erkrankung. Bei gutem Ansprechen auf die Therapie hat die Mehrzahl der Patienten heute eine normale Lebenserwartung.

Abstract

Background

Hairy cell leukemia (HCL) is an indolent B cell lymphoma. Median age at diagnosis is 45–55 years. Men are affected about four times as often as women. Clinical signs are mainly due to cytopenia (neutropenia, thrombocytopenia, and anemia). Further symptoms are splenomegaly and fatigue.

Results

The most common form is designated classic HCL, the uncommon form as HCL variant. Classic HCL is highly sensitive to chemotherapy with purine analogues and remission rates reach 95%. Further effective treatment options are immunotherapy and BRAF inhibitors.

Conclusion

HCL remains a chronic disease. Most patients with a good response to treatment nowadays have a normal life expectancy.

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Authors and Affiliations

  1. Charité Campus Virchow-Klinikum, Medizinische Klinik mit Schwerpunkt Hämatologie, Onkologie und Tumorimmunologie, Ambulantes Gesundheitszentrum der Charité – Universitätsmedizin Berlin, Augustenburger Platz 1, 13344, Berlin, Deutschland

    B. Wörmann

  2. Deutsche Gesellschaft für Hämatologie und Onkologie, Berolinahaus, Alexanderplatz 1, 10178, Berlin, Deutschland

    B. Wörmann

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B. Wörmann gibt an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Wörmann, B. Haarzellleukämie. Onkologe 26, 254–260 (2020). https://doi.org/10.1007/s00761-020-00713-w

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