Treatment of refractory anemia with ring sideroblasts associated with marked thrombocytosis with lenalidomide in a patient testing negative for 5q deletion and JAK2 V617F and MPL W515K/L mutations

  • Ryan Keen Department of Medicine, Medical College of Georgia, Augusta University, Augusta, GA, United States.
  • Jeremy Pantin Department of Hematology/Oncology, Medical College of Georgia, Augusta University, Augusta, GA, United States.
  • Natasha Savage Department of Pathology, Medical College of Georgia, Augusta University, Augusta, GA, United States.
  • Paul M. Dainer | pdainer@augusta.edu Department of Hematology/Oncology, Medical College of Georgia, Augusta University, Augusta, GA, United States.

Abstract

Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematologic malignancy that often results in transfusion dependency and a hypercoagulable state. This rare disease currently lacks formal guidelines for treatment; however, various case reports have demonstrated efficacy in the use of lenalidomide. This immunomodulatory drug has shown promise in patients with 5q deletions, with reports of achieving transfusion independence and normalization of platelet counts. Herein we present the case of a 68-year-old African American woman with RARS-T who tested negative for 5q deletion and JAK2 V617F and MPL W515K/L mutations. Her treatment with lenalidomide therapy resulted in a five-year durable complete clinical response.

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Published
2016-11-02
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Issue
Section
Case Reports
Keywords:
Myelodysplastic-myeloproliferative diseases, Anemia, Refractory, Platelet count, Lenalidomide
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How to Cite
Keen, R., Pantin, J., Savage, N., & Dainer, P. M. (2016). Treatment of refractory anemia with ring sideroblasts associated with marked thrombocytosis with lenalidomide in a patient testing negative for 5q deletion and JAK2 V617F and MPL W515K/L mutations. Hematology Reports, 8(4). https://doi.org/10.4081/hr.2016.6592