title: Recommendations of the SFH (French Society of Haematology) for the diagnosis, treatment and follow-up of hairy cell leukaemia.
abstract: Hairy cell leukaemia (HCL) is a rare haematological malignancy, with approximately 175 new incident cases in France. Diagnosis is based on a careful examination of the blood smear and immunophenotyping of the tumour cells, with a panel of four markers being used specifically to screen for hairy cells (CD11c, CD25, CD103 and CD123). In 2011, the V600E mutation of the BRAF gene in exon 15 was identified in HCL; being present in HCL, it is absent in the variant form of HCL (HCL-v) and in splenic red pulp lymphoma (SRPL), two entities related to HCL. The management of patients with HCL has changed in recent years. A poorer response to purine nucleoside analogues (PNAs) is observed in patients with more marked leukocytosis, bulky splenomegaly, an unmutated immunoglobulin variable heavy chain (IgVH) gene profile, use of VH4-34 or with TP53 mutations. We present the recommendations of a group of 11 experts belonging to a number of French hospitals. This group met in November 2013 to examine the criteria for managing patients with HCL. The ideas and proposals of the group are based on a critical analysis of the recommendations already published in the literature and on an analysis of the practices of clinical haematology departments with experience in managing these patients. The first-line treatment uses purine analogues: cladribine or pentostatin. The role of BRAF inhibitors, whether or not combined with MEK inhibitors, is discussed. The panel of French experts proposed recommendations to manage patients with HCL, which can be used in a daily practice.
authors.affiliation: Haematology Laboratory, Caen University Hospital, 14033, Caen Cedex, France.
authors.forename: Edouard
authors.initials: E
authors.lastname: Cornet
authors.forename: Alain
authors.initials: A
authors.lastname: Delmer
authors.forename: Pierre
authors.initials: P
authors.lastname: Feugier
authors.forename: Francine
authors.initials: F
authors.lastname: Garnache-Ottou
authors.forename: David
authors.initials: D
authors.lastname: Ghez
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
authors.forename: Vincent
authors.initials: V
authors.lastname: Levy
authors.forename: Frédéric
authors.initials: F
authors.lastname: Maloisel
authors.forename: Daniel
authors.initials: D
authors.lastname: Re
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Zini
authors.forename: Xavier
authors.initials: X
authors.lastname: Troussard
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use; D015703:Antigens, CD / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D000964:Antimetabolites, Antineoplastic / Q000008:administration & dosage / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000473:pathology; D014408:Biomarkers, Tumor; D003937:Diagnosis, Differential; D005091:Exons / Q000235:genetics; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D006801:Humans; D016130:Immunophenotyping; D016898:Interferon-alpha / Q000008:administration & dosage / Q000627:therapeutic use; D007943:Leukemia, Hairy Cell / Q000097:blood / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis; D008297:Male; D009154:Mutation; D009363:Neoplasm Proteins / Q000235:genetics; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000188:drug therapy; D011379:Prognosis; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D000069283:Rituximab; D016879:Salvage Therapy; D013160:Splenic Neoplasms / Q000175:diagnosis
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D015703:Antigens, CD; D000951:Antigens, Neoplasm; D000964:Antimetabolites, Antineoplastic; D014408:Biomarkers, Tumor; D016898:Interferon-alpha; D009363:Neoplasm Proteins; D047428:Protein Kinase Inhibitors; D000069283:Rituximab; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf