title: Long-term analysis of the palliative benefit of 2-chlorodeoxyadenosine for myelofibrosis with myeloid metaplasia.
abstract: OBJECTIVE
  Therapeutic splenectomy in myelofibrosis with myeloid metaplasia (MMM) may result in extreme thrombocytosis and leukocytosis and accelerated hepatomegaly. We previously described initial palliative benefit from 2-chlorodeoxyadenosine (2-CdA) in such instances. The purpose of this study is to provide long-term follow-up on the durability of response in the initial cohort and in additional subsequent cases.
  
  
  METHODS
  We retrospectively identified patients with histologically confirmed MMM who had palliative therapy with 2-CdA. Clinical characteristics and information on subsequent clinical course were abstracted at the time of diagnosis of MMM and at initiation of 2-CdA therapy until death.
  
  
  RESULTS
  To date, we have used 2-CdA as palliative therapy in 14 patients with MMM. After a median of four cycles of therapy, responses for hepatomegaly occurred in 56% of patients, thrombocytosis 50%, leukocytosis 55%, and anemia 40%. Cytopenias were frequent but usually transient and without clinical consequence. Responses occurred usually by the second cycle; median duration of response was 6 months (range, 2-19) after completion of 2-CdA therapy.
  
  
  CONCLUSIONS
  This study confirmed relevant and frequently durable palliation of symptoms in about half the patients. 2-CdA is a reasonable palliative option in postsplenectomy patients with MMM who have problematic myeloproliferation.
authors.affiliation: Division of Hematology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Leonardo N
authors.initials: LN
authors.lastname: Faoro
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Ruben A
authors.initials: RA
authors.lastname: Mesa
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D017338:Cladribine / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006402:Hematologic Diseases / Q000139:chemically induced; D006529:Hepatomegaly / Q000139:chemically induced; D006801:Humans; D008297:Male; D008875:Middle Aged; D010166:Palliative Care; D055728:Primary Myelofibrosis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D013156:Splenectomy
chemical_list: D000970:Antineoplastic Agents; D017338:Cladribine