Risk of parenchymal iron overload and its clinical consequences in patients with myelodysplastic syndrome receiving regular blood transfusions and stratified according to WPSS.*
| WPSS risk . | WHO categories . | Clinical features . | Risk of parenchymal iron overload and its clinical consequences . |
|---|---|---|---|
| * Patients with very low WPSS risk are transfusion-independent by definition.52 | |||
| Abbreviations: RARS, refractory anemia with ringed sideroblasts; RA, refractory anemia; RCMD, refractory cytopenia with multilineage dysplasia; RCMD-RS, refractory cytopenia with multilineage dysplasia and ringed sideroblasts; RAEB-1, refractory anemia with excess blasts, type 1; RAEB-2, refractory anemia with excess blasts, type 2. | |||
| Low risk | RARS, or RA, or 5q-syndrome, all with good cytogenetics | Median probability of surviving more than 5 years, low risk of leukemic evolution (in the order of 10–20% at 5 years) | Considerable risk resulting from increased reticuloendothelial iron recycling associated with ineffective erythropoiesis and from long exposition to iron loading. RARS patients appear to be at a particularly high risk of parenchymal organ damage |
| Intermediate risk | RARS or RA with intermediate cytogenetics, RCMD or RCMD-RS with good cytogenetics | Median probability of surviving of about 4 years, risk of leukemic evolution of about 30–40% at 5 years | Parenchymal iron loading and its clinical consequences appear to be a minor problem compared with the high risk of leukemic evolutions. However, they may be relevant in single cases, and should therefore be considered on an individual patient basis. Iron-loaded patients undergoing allogeneic stem cell transplantation may benefit from chelation therapy to reduce the risk of transplant-related mortality. |
| High and very high risk | Various combinations, including also patients with RAEB-1 and RAEB-2 | Median probability of surviving lower than 2 years, risk of leukemic evolution greater than 50% at 5 years | Transfusion iron overload does not represent a clinical problem in the vast majority of these patients. Iron-loaded patients undergoing allogeneic stem cell transplantation may benefit from chelation therapy to reduce the risk of transplant-related mortality |
| WPSS risk . | WHO categories . | Clinical features . | Risk of parenchymal iron overload and its clinical consequences . |
|---|---|---|---|
| * Patients with very low WPSS risk are transfusion-independent by definition.52 | |||
| Abbreviations: RARS, refractory anemia with ringed sideroblasts; RA, refractory anemia; RCMD, refractory cytopenia with multilineage dysplasia; RCMD-RS, refractory cytopenia with multilineage dysplasia and ringed sideroblasts; RAEB-1, refractory anemia with excess blasts, type 1; RAEB-2, refractory anemia with excess blasts, type 2. | |||
| Low risk | RARS, or RA, or 5q-syndrome, all with good cytogenetics | Median probability of surviving more than 5 years, low risk of leukemic evolution (in the order of 10–20% at 5 years) | Considerable risk resulting from increased reticuloendothelial iron recycling associated with ineffective erythropoiesis and from long exposition to iron loading. RARS patients appear to be at a particularly high risk of parenchymal organ damage |
| Intermediate risk | RARS or RA with intermediate cytogenetics, RCMD or RCMD-RS with good cytogenetics | Median probability of surviving of about 4 years, risk of leukemic evolution of about 30–40% at 5 years | Parenchymal iron loading and its clinical consequences appear to be a minor problem compared with the high risk of leukemic evolutions. However, they may be relevant in single cases, and should therefore be considered on an individual patient basis. Iron-loaded patients undergoing allogeneic stem cell transplantation may benefit from chelation therapy to reduce the risk of transplant-related mortality. |
| High and very high risk | Various combinations, including also patients with RAEB-1 and RAEB-2 | Median probability of surviving lower than 2 years, risk of leukemic evolution greater than 50% at 5 years | Transfusion iron overload does not represent a clinical problem in the vast majority of these patients. Iron-loaded patients undergoing allogeneic stem cell transplantation may benefit from chelation therapy to reduce the risk of transplant-related mortality |