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Assessment of the Metabolic Profile of Primary Leukemia Cells
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[Hairy cell leukemia].

Elsa Maitre1, Margaux Wiber1, Edouard Cornet1

  • 1CHU de Caen, laboratoire d'hématologie, 14000 Caen, France.

Presse Medicale (Paris, France : 1983)
|August 27, 2019
PubMed
Summary
This summary is machine-generated.

Hairy cell leukemia (HCL) is identifiable by specific cell markers and a common BRAFV600E mutation. Treatment varies based on mutation status, with purine analogs as first-line and targeted therapies for relapsed or refractory cases.

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Area of Science:

  • Hematology
  • Oncology

Background:

  • Hairy cell leukemia (HCL) is a distinct hematologic malignancy.
  • Morphological identification of hairy cells and expression of markers like CD11c, CD25, CD103, and CD123 are characteristic.
  • The BRAFV600E mutation is present in approximately 80% of HCL cases.

Purpose of the Study:

  • To outline the diagnostic features of HCL.
  • To discuss the differential diagnosis challenges, particularly with HCL variant and other splenic lymphomas.
  • To review current and alternative treatment strategies for HCL.

Main Methods:

  • Morphological analysis of hairy cells.
  • Immunophenotyping using CD markers (CD11c, CD25, CD103, CD123).
  • Genetic testing for BRAFV600E mutation.

Main Results:

  • HCL is characterized by specific hairy cell morphology and immunophenotype.
  • BRAFV600E mutation is a frequent finding, aiding diagnosis and treatment selection.
  • Differential diagnosis can be challenging without the BRAFV600E mutation.

Conclusions:

  • Purine analogs (PNA) with or without anti-CD20 antibodies are standard first-line treatment.
  • BRAF and MEK inhibitors are options for BRAFV600E-mutated relapsed/refractory HCL.
  • Moxetumomab-pasudotox is an alternative for BRAFV600E-negative cases, emphasizing multidisciplinary discussion for complex scenarios.