Case Report

Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy

Table 1

Characteristics of patients reported to have achieved sustained transfusion independence after stopping iron chelation therapy.

CaseAgeFAB or WHO diagnosisKaryotype at diagnosisRisk scoreICTDuration of ICTTime to TI (mo.)Duration of TI (mo.)Duration of TI after stopping ICT (mo.)Other treatmentReference

167 RA del(11)(q22q24)NRDFO13052NR3–362None reported[29]

2 46 RA NormalNRDFO 252024+3–362None reported[29]

3 41 RA NormalNRDFO203620+3–362None reported[29]

4 18 RAEB NormalNRDFO20206+3–362None reported[29]

5 65 MDS +8NRDFO 1550NR3–362Hydrea3[29]

6 61 PMF NRInt.4DFX 20536+22+None[38]

7 63 RARS-TNormalLow5DFX 121.52212+EPO6Current report

AML, acute myelogenous leukemia; DFO, deferoxamine; DFX, deferasirox; Int., intermediate; FAB, French American British; hydrea, hydroxyurea; IPSS, International Prognostic Scoring System; ICT, iron chelation therapy; MDS, myelodysplastic syndrome; mo., months; MPN, myeloproliferative neoplasm; PMF, primary myelofibrosis; RA, refractory anemia; RAEB, refractory anemia with excess blasts; MDS, myelodysplastic syndromes; MPN, myeloproliferative neoplasm; NR, not reported; PMF, primary myelofibrosis; RARS-T, refractory anemia with ring sideroblasts and thrombocytosis; TI, transfusion independence; WHO, World Health Organization; +, indicates ongoing TI.
1TI achieved 15 months after chelation was stopped. 2Duration of TI after stopping chelation for cases 1–5 was reported as a range only. 3Hydroxyurea was given after stopping chelation due to progression to AML. 4Dupriez score (Dupriez et al., 1996 [45]). 5IPSS score for MDS (Greenberg et al., 1997 [1]). 6No response to EPO.