Refractory Anemia with Excess Blasts



Refractory Anemia with Excess Blasts


Carla S. Wilson, MD, PhD









This patient has pancytopenia with 13% circulating blasts image, consistent with RAEB-2. The neutrophil image has dysplastic features with hypogranular cytoplasm and atypical nuclear segmentation.






This 76-year-old man with RAEB-1 has macrocytic anemia, thrombocytopenia, and no circulating blasts. The bone marrow has 7% blasts image and dysplasia of the neutrophilic myeloid lineage image.


TERMINOLOGY


Abbreviations



  • Refractory anemia with excess blasts (RAEB)


Definitions



  • Myelodysplastic syndrome (MDS) with increased blasts or Auer rods


  • 2 categories based on prognosis



    • RAEB-1



      • 2-4% blasts in peripheral blood or


      • 5-9% blasts in bone marrow


    • RAEB-2



      • 5-19% blasts in peripheral blood or


      • 10-19% blasts in bone marrow


      • Auer rods, if present, advance MDS case to RAEB-2


ETIOLOGY/PATHOGENESIS


Hematopoietic Stem Cell Disorder



  • Clonal hematopoietic stem cell disorder


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 35-40% of MDS



      • 15-20% RAEB-1


      • 20% RAEB-2


      • Associated reticulin fibrosis seen in ˜ 10% of MDS; most are RAEB-F (fibrosis)


  • Age



    • Older individuals, primarily > 50 years of age


Presentation



  • Often seek medical attention for symptoms of anemia



    • Fatigue, dyspnea, palpitations, headache, dizziness


  • Other symptoms related to cytopenias



    • Exaggerated bleeding from thrombocytopenia with platelet dysfunction


    • Infection secondary to decreased neutrophils and neutrophil dysfunction


  • Occasional mild hepatomegaly or splenomegaly


Treatment



  • High-risk MDS



    • May be treated similarly to acute myeloid leukemia (AML)



      • Depends partly on tempo of disease progression


    • Intensive chemotherapy with variety of regimens


    • RAEB-2 patients who are candidates for stem cell transplantation



      • Topotecan-cytarabine or idarubicin-cytarabine-based regimens


  • Hematopoietic stem cell transplantation



    • Patient must have low comorbidity scores


    • High upfront treatment-related mortality



      • May want to delay for low-risk or intermediate-risk patients


    • Risk of relapse increases if cytogenetics classified as poor risk


Prognosis



  • 25% 5-year survival rate



    • Patients have progressive bone marrow failure with increasing cytopenias



      • Die from complications such as bleeding and infections


    • Subset progresses to AML



      • 25% of RAEB-1 cases at 5 years


      • 35% (to 50%) of RAEB-2 cases at 5 years


  • Predicted survival based on blast percentage, cytogenetic abnormalities, cytopenias



    • RAEB-1



      • Intermediate-risk prognostic group


      • Median survival is ˜ 16-40 months


    • RAEB-2



      • High-risk group



      • Median survival is ˜ 9-20 months


MICROSCOPIC PATHOLOGY


Cytologic Features

Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Refractory Anemia with Excess Blasts
Premium Wordpress Themes by UFO Themes
%d bloggers like this: