title: Advances in the treatment of hairy-cell leukaemia.
abstract: Hairy-cell leukaemia (HCL) is an uncommon B-cell chronic lymphoproliferative disorder that accounts for about 2% of all leukaemias. Although the disease is generally indolent in its natural course, the majority of patients require treatment for life-threatening infections due to pancytopenia or symptomatic splenomegaly. During the past 20 years, remarkable progress has been made in the treatment of HCL. Since the introduction of interferon-alpha, splenectomy, which was formerly the standard therapy, has been rarely used. With the purine analogues cladribine and pentostatin, response rates are even better than with interferon-alpha and long-lasting remissions can be achieved in most patients. Therefore, these agents are now considered the treatment of choice. Recently, immunotherapeutic approaches which use monoclonal antibodies have increased the number of therapeutic options for HCL and offer promising salvage strategies for patients who relapse or who are refractory to treatment with purine analogues. In this review the different treatment options available are discussed and recommendations for the clinical management of the HCL are summarised.
authors.affiliation: Department of Internal Medicine I, University of Bonn, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Mey
authors.forename: John
authors.initials: J
authors.lastname: Strehl
authors.forename: Marcus
authors.initials: M
authors.lastname: Gorschlüter
authors.forename: Carsten
authors.initials: C
authors.lastname: Ziske
authors.forename: Axel
authors.initials: A
authors.lastname: Glasmacher
authors.forename: Hans
authors.initials: H
authors.lastname: Pralle
authors.forename: Ingo
authors.initials: I
authors.lastname: Schmidt-Wolf
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D015703:Antigens, CD / Q000276:immunology; D000944:Antigens, Differentiation, B-Lymphocyte / Q000276:immunology; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D015815:Cell Adhesion Molecules; D017338:Cladribine / Q000627:therapeutic use; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D037102:Lectins / Q000276:immunology; D007943:Leukemia, Hairy Cell / Q000150:complications / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D018365:Neoplasm, Residual / Q000175:diagnosis; D016609:Neoplasms, Second Primary / Q000139:chemically induced; D010198:Pancytopenia / Q000209:etiology / Q000628:therapy; D015649:Pentostatin / Q000627:therapeutic use; D015375:Receptors, Interleukin-2 / Q000276:immunology; D000069283:Rituximab; D051918:Sialic Acid Binding Ig-like Lectin 2; D013154:Spleen / Q000528:radiation effects; D013156:Splenectomy; D013163:Splenomegaly / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome; D014740:Vidarabine / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D000903:Antibiotics, Antineoplastic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D015703:Antigens, CD; D000944:Antigens, Differentiation, B-Lymphocyte; D000970:Antineoplastic Agents; C497867:CD22 protein, human; D015815:Cell Adhesion Molecules; D016898:Interferon-alpha; D037102:Lectins; D015375:Receptors, Interleukin-2; D051918:Sialic Acid Binding Ig-like Lectin 2; D015649:Pentostatin; D017338:Cladribine; D000069283:Rituximab; D014740:Vidarabine; C024352:fludarabine