title: Classical hairy cell leukemia and its variant: a 17-year retrospective survey in Taiwan Chinese.
abstract: BACKGROUND
  Classical hairy cell leukemia (HCL-C) and its variant (HCL-V) are rare chronic B-cell lymphoproliferative disorders. Only a few reports in Chinese patients are available.
  
  
  METHODS
  We retrospectively reviewed 16 patients with HCL-C and HCL-V in Taiwan over a 17-year period.
  
  
  RESULTS
  Eight were HCL-C and 8 were HCL-V. All HCL accounted for 0.7% of all adult leukemias. Compared to HCL-V, HCL-C was characterized by profound leukopenia, monocytopenia, thrombocytopenia and fewer circulating hairy cells. One HCL-C and 2 HCL-V patients had second malignancies. Seven HCL-C patients achieved hematological remission after splenectomy (n = 1) or 2-chlorodeoxyadenosine (n = 6). Of the 8 HCL-V patients, 6 received splenic irradiation. Only one achieved complete remission and another had partial remission; relapse or disease progression was noted 13.4 or 25.7 months later, respectively. Two of three HCL-V patients who underwent splenectomy had stable disease. All patients with HCL-C were alive while 3 with HCL-V expired. Compared to HCL-C, HCL-V had a significantly shorter leukemia-free survival.
  
  
  CONCLUSIONS
  A relatively higher proportion of HCL-V in all HCL comparing to Westerners is observed. Second malignancies are common. With an inferior outcome and dismal response to most treatment, enrollment in a clinical trial should be considered for HCL-V.
authors.affiliation: Division of Hematology-Oncology, Chang Gung Memorial Hospital, Taipei, Taiwan, ROC.
authors.forename: Hsiao-Wen
authors.initials: HW
authors.lastname: Kao
authors.forename: Po
authors.initials: P
authors.lastname: Dunn
authors.forename: Ming-Chung
authors.initials: MC
authors.lastname: Kuo
authors.forename: Lee-Yung
authors.initials: LY
authors.lastname: Shih
authors.forename: Tung-Liang
authors.initials: TL
authors.lastname: Lin
authors.forename: Jin-Hou
authors.initials: JH
authors.lastname: Wu
authors.forename: Tzung-Chih
authors.initials: TC
authors.lastname: Tang
authors.forename: Hung
authors.initials: H
authors.lastname: Chang
authors.forename: Hong-Cheng
authors.initials: HC
authors.lastname: Wu
authors.forename: Yu-Shin
authors.initials: YS
authors.lastname: Hung
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000145:classification / Q000453:epidemiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013624:Taiwan / Q000453:epidemiology