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ASH Image Bank (2001); doi:10.1182/ashimagebank-2001-100189
Copyright © 2001 by the American Society of Hematology.
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Full Case Study

Myelodysplastic Syndrome: Refractory Anemia with Ringed Sideroblasts (RARS)

James W Vardiman, M.D.

University of Chicago



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  		Figure 1. Refractory anemia with ringed sideroblasts (RARS). Peripheral blood smear (Wright-Giemsa stain). The peripheral blood smear showed normal white blood cells. The neutrophils showed no dysplasia, and the platelets were normal. There were occasional macro-ovalocytes, as well as occasional hypochromic and microcytic red blood cells.

 


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  		Figure 2. Refractory anemia with ringed sideroblasts (RARS). Bone marrow biopsy specimen (H & E stain). The bone marrow biopsy is mildly hypercellular (left panel), and shows erythroid proliferation (right panel). Megakaryocytes are present in normal number, and have normal morphology.

 


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  		Figure 3. Refractory anemia with ringed sideroblasts (RARS). Bone marrow biopsy specimen (H & E stain). This high power view of the biopsy illustrates the erythroid hyperplasia. The immature cells at the arrows are immature erythroid precursors, which are characterized by a round to oval nucleus with vesicular chromatin pattern, and prominent, often comma-shaped nucleoli, and a modest rim of blue-grey (amphophilic) cytoplasm.

 


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  		Figure 4. Refractory anemia with ringed sideroblasts (RARS). Bone marrow aspirate smear (Wright-Giemsa stain). The two panels in this slide illustrate that the megakaryocytes and the granulocytes show normal morphology. The erythroid precursors in this slide do show some mild nuclear/cytoplasmic dyssynchrony (arrow).

 


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  		Figure 5. Refractory anemia with ringed sideroblasts (RARS). Bone marrow aspirate smear (Wright-Giemsa stain). In this slide, there are mild megaloblastoid changes of the erythroid precursors. The morphologic features of the erythroid cells are variable in RARS (see next slide), but the granulocytes have no dysplastic features in this case.

 


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  		Figure 6. Refractory anemia with ringed sideroblasts (RARS). Bone marrow aspirate smear (Wright-Giemsa stain). This composite shows erythroid precursors from RARS. They vary from mildly dyspoietic cells to large, bizarre, multinucleated cells.

 


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  		Figure 7. Refractory anemia with ringed sideroblasts (RARS). Bone marrow aspirate smear (Iron stain). In the aspirate smear stained for iron, iron stores are increased. The higher power views illustrate several ringed sideroblasts, in which at least 10 granules of iron encircle the nucleus. Also illustrated (arrow) is a Type 2 sideroblast. In this cell, there is increased iron depostion, but it is accumulated in the cytoplasm, and does not surround the nucleus.

 


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  		Figure 8. Comparison of features in subtypes of MDS.

 

   Clinical Summary
TOP
 Clinical Summary
Diagnosis
 Discussion
 Differential Diagnosis
 
This 70 year old man was referred with a diagnosis of "chronic anemia" that was first discovered about one year previously at the time of a regular physical examination.  At that time, he had normal serum B12 and serum folic acid levels, but serum ferritin was increased above normal. Liver and thyroid studies were normal. The patient denied alcohol abuse, and was on no regular medications.  Physical examination showed no splenomegaly or hepatomegaly. At the time of referral, WBC = 4.5 x 109/L, Hb = 8.g g/dl, MCV = 107 fl,  RDW = 13.4,  Platelets = 1895 x 109/L.  A bone marrow biopsy and aspirate were performed. Cytogenetic studies were performed from the marrow aspirate, and were reported as 46, XY (100%).

Sex
Male

Age
70

Ethnicity
n/a


   Diagnosis
TOP
 Clinical Summary
 Diagnosis
Discussion
 Differential Diagnosis
 
Myelodysplastic Sydrome, Refractory Anemia with Ringed Sideroblasts (RARS)

 


   Discussion
TOP
 Clinical Summary
 Diagnosis
 Discussion
Differential Diagnosis
 
Definition:  The myelodysplastic syndrome, Refractory Anemia with Ringed Sideroblasts (RARS) is an acquired disorder characterized by an anemia in which 15% or more of the erythroid precursors in the bone marrow are ringed sideroblasts. The ringed sideroblast is defined as an erythroid precursor in which one third or more of the nucleus is encircled by ten or more siderotic granules demonstrated in an iron stain. Myeloblasts account for fewer than 5% of the bone marrow cells. In RARS, dysplasia is limited to the erythroid lineage. Fewer than 10% of the cells of the granulocytic or megakaryocytic lineages demonstrate dysplasia.  Secondary causes of ringed sideroblasts, such as anti-tuberculous drugs and alcohol abuse, must be excluded as possible causes of the dyserythropoiesis.

Synonyms: Pure sideroblastic anemia, acquired idiopathic sideroblastic anemia

Epidemiology: RARS accounts for approximately 10-15% of all cases of MDS. It occurs primarily in older individuals, and more commonly in men.

Etiology: The etiology of  primary (de novo) RARS is unknown.

Clinical features: The presenting clinical features are related to anemia, which usually develops over a prolonged period. The white blood cell count and platelet count are usually normal. Sometimes there is associated thrombocytosis. If the platelet count is more than 1000 x 109/L, a diagnosis of myelodysplastic/myeloproliferative disease should be considered. There is often evidence of iron-overload.

Morphologic features: The red blood cells in the peripheral blood often show anisochromia, i.e., a major population of normochromic and normocytic red blood cells may be mixed with a minor population of hypochromic red blood cells. Other cases have macro-ovalocytes as a minor or major population, or the anemia may be normochromic and normocytic. Blasts are not present in the blood, and monocytes are less than 1 x 109/L. The bone marrow specimen is usually hypercellular or normocellular, and shows erythroid proliferation. Fifteen percent or more of the erythroid precursors are ringed sideroblasts, and often iron stores are substantially increased in amount in the macrophages. Dysplasia is limited mainly to the erythroid precursors. In addition to the ringed sideroblasts, megaloblastic changes, abnormal nuclear lobation or budding, and multinucleation may be seen. Myeloblasts are fewer than 5% of the marrow cells, and there are no Auer rods. Fewer than 10% of the granulocytes or of the megakaryocytes are dysplastic.

Genetic features: Most cases of RARS have normal cytogenetic studies. Fewer than 10% of cases of RARS exhibit clonal chromosomal abnormalities. When they do occur, they are similar to those seen in other patients with MDS, and may include del(7/7q), del(5/5q), or +8.

Clinical outcome: Median survival time for RARS is approximately 6 years. Fewer than 5% will develop acute leukemia.

 


   Differential Diagnosis
TOP
 Clinical Summary
 Diagnosis
 Discussion
 Differential Diagnosis
 
Refractory anemia, Refractory cytopenia with multilineage dysplasia and ringed sideroblasts, Refractory anemia with excess of blasts, Secondary causes of sideroblastic anemia, Hereditary sideroblastic anemia.  (See Table below for comparison of features of MDS with other subtypes of MDS)


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Related ASH-SAP Chapter:space logo
Chapter 9: Myelodysplastic syndrome and overlap syndromes

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Right arrowRelated ASH Education Book Articles
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Copyright © 2001 by the American Society of Hematology.