Chronic myeloid neoplasms
Myelodysplastic syndromes
MDS with single lineage dysplasia


Topic Completed: 1 August 2011

Revised: 3 September 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: MDS with single lineage dysplasia

Nikhil Sangle, M.D.
Page views in 2018: 554
Page views in 2019 to date: 547
Cite this page: Sangle N. MDS with single lineage dysplasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/myeloproliferativeRA.html. Accessed September 18th, 2019.
Definition / general
  • Also called Refractory cytopenia with unilineage dysplasia
  • Ineffective erythropoiesis with erythroid hyperplasia of marrow but low reticulocyte count and anemia
  • Initially termed "refractory" because refractory to iron, folate and vitamin B12 supplements
  • Diagnosis of exclusion; must eliminate other possible causes of anemia (folate / B12 deficiency, drugs, toxins, arsenic, congenital dyserythropoietic anemia)
  • 90 - 95% of patients have a low or intermediate International prognostic scoring system score
  • Occasionally evolves to more aggressive MDS or AML
Laboratory
  • Anemia (Hb < 10g/dL), usually no other cytopenias
Treatment
  • None or transfusion support
  • Combination of all trans retinoic acid, calcitriol and androgens may be effective (Leuk Res 2006;30:935)
Microscopic (histologic) description
  • Peripheral blood: anisocytosis (variation in size), poikilocytosis (variation in shape), normochromic oval macrocytes; usually no neutrophilic or platelet dysplasia; no / rare myeloblasts, monocytes are less than 1 billion/L
  • Bone marrow: hypercellular or normocellular
  • Erythroid: erythroid hyperplasia, may have mild to moderate dysplasia with megaloblastoid features; < 15% ringed sideroblasts
  • Myeloid: myeloblasts < 5% (or call RAEB), no / slight dysplasia
  • Megakaryocytes: no / slight dysplasia
  • Patients with 2 - 4% blasts in peripheral blood and < 5% blasts in bone marrow should be classified as RAEB-1
Microscopic (histologic) images

Images hosted on PathOut server:

Bone marrow biopsy:

Slightly hypercellular for a 53 year old man,
marked erythroid hyperplasia,
prominent megakaryocytes but no increase in their number



Bone marrow smear:

Two postmitotic erythroid precursors
(upper left) with internuclear chromatin bridging

Molecular / cytogenetics description
  • No specific abnormality, 70% have normal karyotype (Atlas of Genetics and Cytogenetics)
  • Refractory neutropenia and refractory thrombocytopenia are rare and caution needs to be exercised in making these diagnoses (these fall into the category of Refractory cytopenia with unilineage dysplasia)
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