What is the best treatment approach for an 87-year-old patient with chronic anemia, myelodysplastic syndrome, and sideroblastic anemia?

Treatment Approach for 87-Year-Old with Chronic Anemia, MDS, and Sideroblastic Anemia

For an 87-year-old patient with chronic anemia, myelodysplastic syndrome, and sideroblastic anemia, supportive care with regular red blood cell transfusions to maintain hemoglobin >10 g/dL is the most appropriate treatment approach to improve quality of life and reduce morbidity.

Assessment of Laboratory Values

• The patient's labs show severe macrocytic anemia (RBC 2.14, Hgb 7.1, HCT 23%, MCV 107.8) with anisocytosis (RDW 35.4), consistent with MDS and sideroblastic anemia 1
• The macrocytosis (MCV 107.8) and elevated RDW are typical findings in sideroblastic anemia associated with MDS 2

Treatment Algorithm for Elderly Patient with MDS and Sideroblastic Anemia

First-Line Treatment Options:

1. Supportive Care with RBC Transfusions

   • Recommended as primary treatment for frail elderly patients with MDS 1
   • Administer transfusions at hemoglobin threshold of at least 8 g/dL, or 9-10 g/dL in case of comorbidities or poor functional tolerance 1
   • Sufficient RBC concentrates should be given to increase hemoglobin >10 g/dL to limit effects of chronic anemia on quality of life 1

2. Erythropoiesis-Stimulating Agents (ESAs)

   • Consider EPO alpha at higher doses (40,000-60,000 units 1-3 times weekly) 1
   • Most effective when serum EPO levels are ≤500 mU/mL and transfusion requirements are limited 1
   • Response rates are generally lower in sideroblastic anemia compared to other MDS subtypes 1

3. ESA plus G-CSF Combination

   • Addition of G-CSF (1-2 mcg/kg daily or 1-3 times weekly) can enhance response in patients with ring sideroblasts 1, 3
   • Particularly effective in MDS with ring sideroblasts, with response rates of up to 75% 3

Second-Line Treatment Options:

1. Luspatercept

   • Recommended after ESA failure in transfusion-dependent MDS with ring sideroblasts 1, 4
   • Targets ineffective erythropoiesis through inhibition of TGF-beta pathway 4
   • May reduce transfusion requirements for more than a year 5

2. Combination of Luspatercept with ESAs

   • May be considered in patients who fail monotherapy 6
   • Stimulates both early and late-stage erythropoiesis 6

3. Iron Chelation Therapy

   • Consider in patients who have received 20-60 RBC concentrates or if serum ferritin rises above 1000-2500 U/L 1
   • Deferasirox is the most widely used oral chelator but cannot be used in patients with renal failure 1, 7, 5
   • Strongly recommended in patients with major iron overload (significantly reduced cardiac T2* on MRI) 1

Special Considerations for This Patient

• At 87 years old with chronic anemia and MDS/sideroblastic anemia, the patient falls into the "frail patient" category 1
• Intensive therapies like allogeneic stem cell transplantation or AML-like chemotherapy are not appropriate due to age and frailty 1
• Primary goal should be symptom management, quality of life improvement, and minimizing treatment-related toxicity 1

Monitoring Recommendations

• Regular blood counts to detect worsening cytopenias 1
• Monitor for signs of iron overload, especially if receiving regular transfusions 1, 5
• Assess cardiac function periodically, as heart iron overload is a risk after receiving approximately 70-80 RBC concentrates 1

Common Pitfalls to Avoid

• Underdosing ESAs - higher doses than those used for renal anemia are required (40,000-60,000 units vs standard doses) 1
• Failing to verify iron repletion before starting ESA therapy 1
• Neglecting iron chelation in regularly transfused patients, which can lead to organ damage 1, 5
• Overlooking psychosocial support and patient support group connections 1

In this elderly patient with MDS and sideroblastic anemia, a supportive care approach with regular RBC transfusions to maintain adequate hemoglobin levels represents the most appropriate treatment strategy, with consideration of ESAs with G-CSF if the patient has low serum EPO levels and limited transfusion requirements.