Hairy cell leukemia (HZL)

  • Method:
  • Anticoagulant:
  • Recommendation:
  • Method:
    Cytomorphology
  • Anticoagulant:
    EDTA
  • Recommendation:
    obligatory
  • Method:
    Immunophenotyping
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory
  • Method:
    Chromosome analysis
  • Anticoagulant:
  • Recommendation:
    no
  • Method:
    FISH
  • Anticoagulant:
  • Recommendation:
    no
  • Method:
    Molecular genetics
  • Anticoagulant:
    EDTA or Heparin
  • Recommendation:
    obligatory

Based on the current guidelines and the current state of research, there are different diagnostic recommendations for patients with hairy cell leukemia. We have summarized the most important information about the classification and diagnostic methods at MLL. In addition, we provide further links on prognosis and therapy in hairy cell leukemia, so that you can inform yourself in more detail.

HZL: Classification

According to the WHO classification 2022, hairy cell leukemia (HCC) belongs to the mature B-cell neoplasms and is a rare disease that is usually indolent. Clinically, it usually presents with marked splenomegaly and pancytopenia. Characteristic is the occurrence of hairy cells with fine, hair-shaped cytoplasmic extensions. The central molecular genetic finding today is the BRAF mutation V600E, which is detectable in virtually all patients with classic hairy cell leukemia and is useful for differentiation from other indolent non-Hodgkin lymphomas (WHO 2022).

The variant form of hairy cell leukemia (HZL-v) previously classified by WHO in 2017 (Swerdlow et al. 2017) is listed in WHO Classification 2022 as a separate entity named "splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN)" due to its distinct differences from HZL and the unrelated biology of the two diseases. The term variant form of hairy cell leukemia continues to be accepted as an alternative to SBLPN (WHO 2022).

HZL: Diagnostic methods and their relevance

HZL: Prognosis

Classical hairy cell leukemia has a good prognosis in the majority of patients and a normal life expectancy for about 70% of patients. The response to therapy is crucial, because patients who achieve a complete remission have a significantly better prognosis than patients with partial remission (Onkopedia guideline HZL 2022). Controversy still exists as to whether the risk of secondary tumors is increased (Maitre et al. 2019).

HZL: Therapy

Guidelines on treatment strategies are available, for example, from the National Comprehensive Cancer Network (NCCN), from Grever et al. 2017 and in the Onkopedia guideline Hairy Cell Leukemia (HZL). Mendez-Hernandez et al. 2023 also provide an overview of current and potential future treatment options (Mendez-Hernandez et al. 2023).

Status: November 2023

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