PMID- 19557769
OWN - NLM
STAT- MEDLINE
DCOM- 20100415
LR  - 20100322
IS  - 1099-1069 (Electronic)
IS  - 0278-0232 (Linking)
VI  - 28
IP  - 1
DP  - 2010 Mar
TI  - Improved survival in red blood cell transfusion dependent patients with primary 
      myelofibrosis (PMF) receiving iron chelation therapy.
PG  - 40-8
LID - 10.1002/hon.902 [doi]
AB  - Many patients with primary myelofibrosis (PMF) become red blood cell (RBC) 
      transfusion dependent (TD), risking iron overload (IOL). Iron chelation therapy 
      (ICT) may decrease the risk of haemosiderosis associated organ dysfunction, 
      though its benefit in PMF is undefined. To assess the effect of TD and ICT on 
      survival in PMF, we retrospectively reviewed 41 patients. Clinical data were 
      collected from the database and by chart review. The median age at PMF diagnosis 
      was 64 (range 43-86) years. Median white blood cell (WBC) count at diagnosis was 
      7.6 (range 1.2-70.9) x 10(9)/L; haemoglobin 104 (62-145) G/L; platelets 300 
      (38-2088) x 10(9)/L. Lille, Strasser, Mayo and International Prognostic System 
      (IPS) scores were: low risk, n = 15, 8, 11, 3; intermediate, n = 15, 19, 9, 16; 
      high, n = 5, 11, 5, 7; respectively. Primary PMF treatment was: supportive care, 
      n = 23; hydroxyurea, n = 10; immunomodulatory, n = 4; splenectomy, n = 2. Sixteen 
      patients were RBC transfusion independent (TI) and 25 TD; of these 10 received 
      ICT for a median of 18.3 (0.1-117) months. Pre-ICT ferritin levels were a median 
      of 2318 (range 263-8400) and at follow up 1571 (1005-3211 microg/L (p = 0.01). In 
      an analysis of TD patients, factors significant for overall survival (OS) were: 
      WBC count at diagnosis (p = 0.002); monocyte count (p = 0.0001); Mayo score (p = 
      0.05); IPS (p = 0.02); number of RBC units (NRBCU) transfused (p = 0.02) and ICT 
      (p = 0.003). In a multivariate analysis, significant factors were: NRBCU (p = 
      0.001) and ICT (p = 0.0001). Five year OS for TI, TD-ICT and TD-NO ICT were: 100, 
      89 and 34%, respectively (p = 0.003). The hazard ratio (HR) for receiving >20 
      RBCU was 7.6 (95% Confidence Intervals [CI] 1.2-49.3) and for ICT was 0.15 
      (0.03-0.77). In conclusion, 61% of PMF patients developed RBC-TD which portended 
      inferior OS; however patients receiving ICT had comparatively improved OS, 
      suggesting a clinical benefit. Prospective studies of IOL and the impact of ICT 
      in PMF are warranted.
FAU - Leitch, Heather A
AU  - Leitch HA
AD  - Division of Hematology, St. Paul's Hospital and the University of British 
      Columbia, 440-1144 Burrard St., Vancouver, BC, V6T 1Z6, Canada. 
      hleitch@providencehematology.com
FAU - Chase, Jocelyn M
AU  - Chase JM
FAU - Goodman, Trisha A
AU  - Goodman TA
FAU - Ezzat, Hatoon
AU  - Ezzat H
FAU - Rollins, Meaghan D
AU  - Rollins MD
FAU - Wong, Dominic H C
AU  - Wong DH
FAU - Badawi, Maha
AU  - Badawi M
FAU - Leger, Chantal S
AU  - Leger CS
FAU - Ramadan, Khaled M
AU  - Ramadan KM
FAU - Barnett, Michael J
AU  - Barnett MJ
FAU - Foltz, Lynda M
AU  - Foltz LM
FAU - Vickars, Linda M
AU  - Vickars LM
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Hematol Oncol
JT  - Hematological oncology
JID - 8307268
RN  - 0 (Iron Chelating Agents)
RN  - 9007-73-2 (Ferritins)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Chelation Therapy/*mortality
MH  - Erythrocyte Transfusion/*mortality
MH  - Female
MH  - Ferritins/*metabolism
MH  - Humans
MH  - Iron Chelating Agents/*therapeutic use
MH  - Iron Overload/drug therapy/etiology/*mortality
MH  - Male
MH  - Middle Aged
MH  - Primary Myelofibrosis/blood/*mortality/therapy
MH  - Prognosis
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Treatment Outcome
EDAT- 2009/06/27 09:00
MHDA- 2010/04/16 06:00
CRDT- 2009/06/27 09:00
PHST- 2009/06/27 09:00 [entrez]
PHST- 2009/06/27 09:00 [pubmed]
PHST- 2010/04/16 06:00 [medline]
AID - 10.1002/hon.902 [doi]
PST - ppublish
SO  - Hematol Oncol. 2010 Mar;28(1):40-8. doi: 10.1002/hon.902.