title: Red Blood Cell Transfusion Dependency and Hyperferritinemia Are Associated with Impaired Survival in Patients Diagnosed with Myelodysplastic Syndromes: Results from the First Polish MDS-PALG Registry.
abstract: BACKGROUND
  Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders characterized by ineffective hematopoiesis, cytopenias and a risk of progression to acute myeloid leukemia (AML). Anemia is the most frequent cytopenia diagnosed in patients with MDS. Regular RBC transfusions are the only treatment option for about 40% of patients. Transfusion-dependent patients develop secondary iron overload. The influence of serum ferritin (SF) concentration on survival and acute myeloid leukemia transformation in MDS patients remains controversial. The data for the Central European population is scarce and so far there is no description for Poland.
  
  
  OBJECTIVE
  The aim of this study was to perform a retrospective analysis of the relationship of SF concentration with red blood cell transfusion dependency, survival and transformation to acute myeloid leukemia.
  
  
  METHODS
  We retrospectively evaluated the data of the 819 MDS patients (58% male; median age 70 years) included in the MDS Registry of the MDS Section of the Polish Adult Leukemia Group (PALG).
  
  
  RESULTS
  Analyses were performed on 190 patients diagnosed with MDS, maximal 6 months before inclusion to the registry in order to avoid selection bias (a shorter survival of higher risk MDS patients). Patients with hyperferritinemia higher than 1000 ng/L vs. patients with SF concentration lower than 1000 ng/L had a median survival of 320 days vs. 568 days, respectively (p log-rank = 0.014). The following factors were found to significantly worsen survival: RBC-transfusion dependence (p = 0.0033; HR 2.67L), platelet transfusion dependence (p = 0.0071; HR 3.321), hemoglobin concentration lower than 10 g/dL (p = 0.0036; HR 2.97), SF concentration higher than 1000 ng/L (p = 0.0023; HR = 2.94), platelet count lower than 10 G/L (p = 0.0081 HR = 5.04), acute leukemia transformation (p = 0.0081; HR 1.968).
  
  
  CONCLUSIONS
  Taking into account the relatively low number of patients in previous studies exploring hyperferritinemia in MDS, the results of the first Polish MDS Registry provide important insights. Hyperferritinemia higher than 1000 ng/L can be an important indicator of poor prognosis in MDS.
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Waszczuk-Gajda
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Mądry
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Rafał
authors.initials: R
authors.lastname: Machowicz
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Drozd-Sokołowska
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Silesian Medical University, Katowice, Poland.
authors.forename: Beata
authors.initials: B
authors.lastname: Stella-Hołowiecka
authors.affiliation: Department of Hematology and Bone Marrow Transplantation, Medical University of Gdańsk, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Mital
authors.affiliation: Department of Hematology, Holycross Cancer Center, Kielce, Poland.
authors.forename: Agata
authors.initials: A
authors.lastname: Obara
authors.affiliation: Department of Hematology, Medical University of Lodz, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Szmigielska-Kapłon
authors.affiliation: Department of Hematology, Institute of Hematology and Transfusiology, Warszawa, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Sikorska
authors.affiliation: Department of Hematology and Internal Diseases, Military Institute of Medicine, Warszawa, Poland.
authors.forename: Edyta
authors.initials: E
authors.lastname: Subocz
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Wiesław W
authors.initials: WW
authors.lastname: Jędrzejczak
authors.affiliation: Department of Hematology, Oncology and Internal Medicine, Medical University of Warsaw, Poland.
authors.forename: Jadwiga
authors.initials: J
authors.lastname: Dwilewicz-Trojaczek
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D019190:Iron Overload / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000401:mortality* / Q000628:therapy*; D011044:Poland; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D005293:Ferritins
keywords: hyperferritinemia; myelodysplastic syndrome; red blood cell transfusion dependence; serum ferritin concentration; transformation to acute myeloid leukemia