Case Presentation: Myelodysplastic Syndrome Case Presentation

Amer Zeidan, MBBS, Yale University, New Haven, Connecticut, highlights his treatment choices for a 74-year-old woman diagnosed with lower-risk myelodysplastic syndrome (MDS). 

This patient is a 74-year-old woman diagnosed with lower-risk myelodysplastic syndrome (MDS). Her initial presentation was a common presentation for many lower-risk MDS patients, which is a presentation with symptoms of anemia.  She had progressive dyspnea on exertion, including when she goes up stairs, and some fatigue. Her primary care doctor did blood work, which showed that her hemoglobin was low at 7.5 [grams (g)/ deciliter (dL)]. She had normal white cell count with differential, as well as a normal platelet count. 

The primary care physician ran a standard test looking for B12, folate nutritional deficiencies, et cetera. Nothing came to explain the anemia, so the patient was referred to a hematologist. The hematologist analyzed her blood smear and saw that there was some evidence of dysplasia with abnormal-looking white blood cells as well as evidence of macrocytes, or large red blood cells.

The patient eventually underwent a bone marrow biopsy, and she was found to have myelodysplastic syndrome without excess blasts. The blast count was 2%. She had a normal karyotype. Molecular assessment showed that she had SF3B1 mutation as well as a TET2 mutation. Importantly, the pathology, in addition to revealing single-lineage dysplasia and evidence of fibrosis, also reported the presence of ring sideroblasts. The patient also had an erythropoietin level checked, and that was 80 [International Units per liter (IU/L)].

The patient eventually was started on erythropoiesis-stimulating agents after it was decided that she had a lower-risk MDS using the International Prognostic Scoring System (IPSS) and the Revised International Prognostic Scoring (IPSS-R) systems. Bone marrow transplant was not indicated at this point. She was in good health. She had diabetes, hypertension, and osteoarthritis, but she was functional.

She started on the erythropoiesis-stimulating agents (ESAs). She started on darbepoetin alfa (Aranesp). She was given a dose of 500 micrograms every 3 weeks subcutaneously. She initially responded to this therapy. Her hemoglobin rose to between 9 and 10 g/dL and she did not need transfusions for almost 1.5 years. However, subsequently her hemoglobin started to drop to around 6.5 g/dL, and she needed to start red blood cell transfusions. 

In this case, what second-line option would be best to consider for this patient?