From the Guidelines
Causes of Diffuse Edema in MDS Patients
The diffuse edema experienced by your Myelodysplastic Syndrome (MDS) patient may be attributed to fluid overload resulting from transfusion dependence 1. This condition is common in MDS patients, as more than 90% present with anemia at diagnosis, and 60% experience severe anemia during the course of their disease 1.
Transfusion Dependence and Iron Overload
Transfusion dependence leads to iron overload, which can cause cardiac dysfunction, resulting in fluid retention and edema 1. Iron overload is defined as a serum ferritin level >1,000 ng/mL 1. The development of secondary iron overload significantly worsens survival, with a 30% increase in hazard for every 500 ng/mL increase in serum ferritin above the threshold 1.
Monitoring and Management of Iron Overload
To manage iron overload, serum ferritin levels and associated organ dysfunction (heart, liver, and pancreas) should be monitored 1. The goal is to decrease ferritin levels to less than 1000 mcg/L 1. Iron chelation therapy with agents like deferoxamine or deferasirox may be considered for patients with low or intermediate-1 risk disease who have received or are anticipated to receive more than 20 RBC transfusions 1.
Key Considerations
- RBC transfusion requirement is a negative prognostic factor for MDS patients 1.
- Iron chelation therapy can reverse some consequences of iron overload, including improvement in cardiac iron content and transfusion independence in some patients 1.
- Monitoring of serum ferritin levels, number of RBC transfusions, and organ dysfunction is essential for managing iron overload 1.
- Deferasirox has been associated with adverse events, including gastrointestinal disturbances, increased serum creatinine, and rare but serious events like acute renal failure or hepatic failure 1.
Treatment Recommendations
- Consider iron chelation therapy with deferoxamine or deferasirox for patients with low or intermediate-1 risk disease who have received or are anticipated to receive more than 20 RBC transfusions 1.
- Monitor serum ferritin levels and organ dysfunction regularly 1.
- Aim to decrease ferritin levels to less than 1000 mcg/L 1.
From the Research
Diffuse Edema in Myelodysplastic Syndrome (MDS) Patients
- Diffuse edema can be a symptom of various complications associated with MDS, including paraneoplastic autoimmune vasculitis 2, autoinflammatory lymphedema 3, and autoimmune phenomena 4.
- MDS patients may experience edema due to underlying conditions such as membranous glomerulonephritis 5 or IgA nephropathy 6, which can lead to nephrotic syndrome and subsequent edema.
- Autoimmune phenomena, including vasculitis and glomerulonephritis, can cause edema in MDS patients 4.
- Treatment with immunosuppressive agents, such as corticosteroids and azacytidine, may improve edema and other symptoms associated with MDS-related autoimmune phenomena 2, 3, 4.
Potential Causes of Diffuse Edema
- Paraneoplastic autoimmune vasculitis: a rare but potentially deadly complication of MDS, characterized by diffuse alveolar hemorrhage and edema 2.
- Autoinflammatory lymphedema: a condition associated with MDS, characterized by bilaterally symmetrical, non-pitting edema on the lower legs 3.
- Membranous glomerulonephritis: a rare condition that can cause nephrotic syndrome and edema in MDS patients 5.
- IgA nephropathy: a condition that can cause renal dysfunction and edema in MDS patients 6.
Treatment and Management
- Immunosuppressive therapy, such as corticosteroids and azacytidine, may be effective in treating edema and other symptoms associated with MDS-related autoimmune phenomena 2, 3, 4.
- Treatment of underlying conditions, such as membranous glomerulonephritis and IgA nephropathy, may also improve edema and other symptoms 5, 6.