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 J Keen, Jeremy Mark Pantin, Natasha Marie Savage, Paul M Dainer

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish (US)
Article number6592
Pages (from-to)48-50
Number of pages3
JournalHematology Reports
Volume8
Issue number4
DOIs
StatePublished - Nov 2 2016

Fingerprint

Refractory Anemia
Thrombocytosis
Mutation
Hematologic Neoplasms
Rare Diseases
Platelet Count
African Americans
Therapeutics
Guidelines
Pharmaceutical Preparations
lenalidomide

Keywords

  • Anemia
  • Lenalidomide
  • Myelodysplastic-myeloproliferative diseases
  • Platelet count
  • Refractory

ASJC Scopus subject areas

  • Hematology

Cite this

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title = "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",
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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AU - Savage, Natasha Marie

AU - Dainer, Paul M

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N2 - 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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