title: Impaired interferon alpha response in hairy cell leukemia is corrected by therapy with 2-chloro-2'-deoxyadenosine: implications for susceptibility to opportunistic infections.
abstract: Patients with hairy cell leukemia (HCL) are susceptible to opportunistic intracellular infections, suggesting defects in cellular immunity. Prior studies have indicated an association between failure of IFN-alpha generation by peripheral blood mononuclear cells (MNC) and susceptibility to such infections. We here present results on IFN-alpha generation in HCL patients pre- and post-therapy. Prior to treatment with 2-chloro-2'-deoxyadenosine (CdA), MNC from 24 HCL patients with active disease produced little or not IFN-alpha (geometric mean < 40 IU/ml) compared with controls (n = 140, geometric mean 1730 IU/ml, p < 0.0005). After treatment with CdA, IFN-alpha generation was studied in 16 patients, with a geometric mean value of 650 IU/ml (p < 0.0005 compared with pre-CdA levels). The severe depression of IFN-alpha generation improved progressively following CdA therapy-induced clinical remission. We propose that deficiency of IFN-alpha production may play a role in the susceptibility to intracellular infections of patients with active HCL.
authors.affiliation: Long Island Jewish Medical Center, Long Island Campus for the Albert Einstein College of Medicine, New Hyde Park, NY 11042.
authors.forename: F P
authors.initials: FP
authors.lastname: Siegal
authors.forename: M
authors.initials: M
authors.lastname: Shodell
authors.forename: K
authors.initials: K
authors.lastname: Shah
authors.forename: D
authors.initials: D
authors.lastname: Drake
authors.forename: M
authors.initials: M
authors.lastname: Hoffman
authors.forename: A
authors.initials: A
authors.lastname: Sawitsky
authors.forename: D
authors.initials: D
authors.lastname: Janson
authors.forename: P
authors.initials: P
authors.lastname: Fitzgerald-Bocarsly
authors.forename: K R
authors.initials: KR
authors.lastname: Rai
mesh_terms: D000328:Adult; D000368:Aged; D017338:Cladribine / Q000627:therapeutic use*; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000096:biosynthesis*; D007943:Leukemia, Hairy Cell / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D007963:Leukocytes, Mononuclear / Q000378:metabolism; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D009894:Opportunistic Infections / Q000150:complications / Q000276:immunology*; D012074:Remission Induction; D013156:Splenectomy; D016176:T-Lymphocyte Subsets
chemical_list: D016898:Interferon-alpha; D017338:Cladribine