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Chronic Myeloid Neoplasms

Myelodysplastic syndromes

Refractory anemia


Reviewer: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 12 August 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● 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
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● Anemia (Hb<10g/dL), usually no other cytopenias

Treatment
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● None or transfusion support
● Combination of all-trans retinoic acid, calcitriol, and androgens may be effective (Leuk Res 2006;30:935)

Micro description
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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

Micro images
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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 post-mitotic erythroid precursors (upper left) with internuclear chromatin bridging

Various images

Cytogenetics
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● 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)

End of Chronic Myeloid Neoplasms > Myelodysplastic syndromes > Refractory anemia


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