title: Extramedullary leukemia adversely affects hematologic complete remission rate and overall survival in patients with t(8;21)(q22;q22): results from Cancer and Leukemia Group B 8461.
abstract: OBJECTIVE
  To examine the prognostic significance of extramedullary leukemia (EML) at presentation in patients with t(8;21)(q22;q22) karyotype.
  
  
  METHODS
  Consecutive patients with t(8;21) treated on Cancer and Leukemia Group B de novo acute myeloid leukemia (AML) treatment studies were examined for the presence of EML (granulocytic sarcoma, subcutaneous nodules, leukemia cutis, or meningeal leukemia) at initial presentation. Clinical features and outcome of t(8;21) patients with and without EML were compared.
  
  
  RESULTS
  Of 84 patients with t(8;21), eight (9.5%) had EML manifesting as granulocytic sarcoma (five paraspinal, one breast, and one subcutaneous) or symptomatic meningeal leukemia (n = 1). The pretreatment prognostic variables of t(8;21) patients with and without EML were similar. The hematologic complete remission (CR) rate for t(8;21) patients with EML was 50% versus 92% for those without EML (P=.006). The median CR duration for EML patients was 14.7 months. Patients with EML had a shorter survival (P = 0.002, median 5.4 months versus 59.5 months). This poor outcome may relate to inadequate local (radiation or intrathecal) therapy for patients with spinal or meningeal EML, resulting in residual/recurrent EML following induction chemotherapy (n = 2) or at relapse (n = 1) and permanent neurologic deficits (n = 4). Only one of the EML patients received high-dose cytarabine (HDAC) intensification; this is the only EML patient remaining alive in CR.
  
  
  CONCLUSIONS
  Patients with t(8;21) and EML have a low CR rate and overall survival. An aggressive local and systemic induction therapy should be considered for this patient subset. The effectiveness of HDAC intensification in t(8;21) patients with EML is uncertain and warrants further study.
authors.affiliation: Cancer and Leukemia Group B, Chicago, IL, USA. john_c.byrd@wramaa.chcs.amedd.army.mil
authors.forename: J C
authors.initials: JC
authors.lastname: Byrd
authors.forename: R B
authors.initials: RB
authors.lastname: Weiss
authors.forename: D C
authors.initials: DC
authors.lastname: Arthur
authors.forename: D
authors.initials: D
authors.lastname: Lawrence
authors.forename: M R
authors.initials: MR
authors.lastname: Baer
authors.forename: F
authors.initials: F
authors.lastname: Davey
authors.forename: E S
authors.initials: ES
authors.lastname: Trikha
authors.forename: A J
authors.initials: AJ
authors.lastname: Carroll
authors.forename: R
authors.initials: R
authors.lastname: Tantravahi
authors.forename: M
authors.initials: M
authors.lastname: Qumsiyeh
authors.forename: S R
authors.initials: SR
authors.lastname: Patil
authors.forename: J O
authors.initials: JO
authors.lastname: Moore
authors.forename: R J
authors.initials: RJ
authors.lastname: Mayer
authors.forename: C A
authors.initials: CA
authors.lastname: Schiffer
authors.forename: C D
authors.initials: CD
authors.lastname: Bloomfield
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001943:Breast Neoplasms / Q000235:genetics; D002891:Chromosomes, Human, Pair 21; D002898:Chromosomes, Human, Pair 8; D005260:Female; D006801:Humans; D007621:Karyotyping; D007938:Leukemia / Q000188:drug therapy* / Q000235:genetics*; D015470:Leukemia, Myeloid, Acute / Q000188:drug therapy* / Q000235:genetics*; D008297:Male; D008577:Meningeal Neoplasms / Q000235:genetics; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000188:drug therapy* / Q000235:genetics*; D011237:Predictive Value of Tests; D011379:Prognosis; D012074:Remission Induction; D016019:Survival Analysis; D014178:Translocation, Genetic; D016896:Treatment Outcome