Laboratoire d'hématologie, CHU Côte de Nacre, Caen, Registre régional des hémopathies malignes de Basse-Normandie, CHU Côte de Nacre, Caen, UPRES EA 3919, Université de Caen Basse-Normandie et UFR de Médecine de Caen, Caen
Hairy-cell leukemia (HCL) is a rare hematologic malignancy. HCL diagnosis can be difficult and the variant form of HCL (HCL-V) has similarities with B-cell prolymphocytic leukemia or splenic marginal zone lymphoma. We describe the different features of HCL, particularly typical HCL (HCL-C) and HCL-V. HCL prognosis was transformed by the introduction of purine nucleoside analogues such as Cladribine or Pentostatin. Both agents are highly effective and the survival of HCL patients is simlilar to that of the general population. The study of residual disease is important and the negativity could mean cure of the disease. The patients refractory to purine nucleoside analogues could benefit treatments with monoclonal antibodies.