From the Guidelines
Causes of Diffuse Edema in MDS Patients
The diffuse edema experienced by your Myelodysplastic Syndromes (MDS) patient may be attributed to fluid overload resulting from transfusion dependence. Many MDS patients require frequent blood transfusions to manage anemia, leading to an excess of iron and fluid in the body. As a result, the body may retain fluid, causing edema, particularly in dependent areas such as the legs and ankles.
Transfusion Dependence and Iron Overload
According to the study by 1, more than 90% of MDS patients will present with anemia at the time of their diagnosis, and 60% of all MDS patients will experience severe anemia at some point during the course of their disease. The development of transfusion dependency in MDS patients is associated with a shorter patient survival and an increased risk of conversion to acute myeloid leukemia. Additionally, secondary iron overload can significantly worsen survival, with a 30% increase in hazard for every 500 ng/mL of increase in serum ferritin above the threshold of 1,000 ng/mL.
Monitoring and Management of Iron Overload
The study by 1 recommends monitoring serum ferritin levels and associated organ dysfunction (heart, liver, and pancreas) in patients with chronic RBC transfusion need. The goal is to decrease ferritin levels to less than 1,000 mcg/L. Iron chelation therapy with deferoxamine or deferasirox may be considered in patients with low or intermediate-1 risk disease who have received or are anticipated to receive more than 20 RBC transfusions.
Key Points
- Transfusion dependence is a common issue in MDS patients, leading to fluid overload and iron overload.
- Iron overload can cause organ dysfunction and worsen survival in MDS patients.
- Monitoring serum ferritin levels and associated organ dysfunction is crucial in managing iron overload.
- Iron chelation therapy may be effective in reducing ferritin levels and improving organ function in MDS patients.
Treatment Options
- Deferoxamine: an iron chelator that can be administered subcutaneously.
- Deferasirox: an oral iron chelator that can be used to reduce ferritin levels.
- Transfusion therapy: should be tailored to individual patient needs, with the goal of improving quality of life and avoiding anemia-related symptoms.
From the Research
Causes of Diffuse Edema in Myelodysplastic Syndromes (MDS) Patients
- Autoimmune phenomena are common in patients with MDS, and can manifest as an acute systemic vasculitic syndrome, characterized by skin vasculitis, fever, arthritis, and sometimes associated with pulmonary infiltrates and peripheral edema 2
- MDS can be associated with various renal disorders, including membranous glomerulonephritis (MGN) and IgA nephropathy (IgAN), which can cause edema in the lower extremities 3, 4
- Paraneoplastic autoimmune vasculitis can occur in MDS patients, leading to diffuse alveolar hemorrhage and potentially causing edema 5
- Autoinflammatory lymphedema can also occur in MDS patients, causing non-pitting edema on the lower legs 6
Possible Underlying Mechanisms
- Immune abnormalities, such as hypergammaglobulinemia and a positive FANA, are common in MDS patients and may contribute to the development of autoimmune phenomena and edema 2
- The pathogenesis of MDS and associated renal disorders, such as IgAN, may involve immune and bone marrow abnormalities 4