REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS

Case History

The patient is a 70 year old man with a history of pancytopenia. He had persistent cytopenia requiring 3 units of blood in December of 2011. His last CBC showed a white cell count of 2.3, hemoglobin of 7.5, and hematocrit of 23 and a platelet count of 80000 with absolute neutrophil count of 1.2. A bone marrow aspirate and biopsy was performed on the right iliac crest.

Microscopic findings

Bone marrow aspirate shows marked erythroid hyperplasia with megaloblastic maturation. Occasional dyserythropoietic precursors are also identified. Myeloid cells show progressive maturation with no increase in the number of blast cells. Many dwarf,monolobated megakaryocytes are noted on scan. An iron stain shows many ringed sideroblats comprising 70% of erythroblastic population.

Diagnosis

REFRACTORY CYTOPENIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS

Discussion

Refractory anemia with multilineage dysplasia is defined as a type of myelodysplatic syndromewith one or more cytopenias and dysplastic changes in two or more of the lineages. There are <1%blasts in the peripheral blood and <5% blasts in the bone marrow. Most patients present with signs and symptoms related to bone marrow failure. Clonal genetic abnormalities including trisomy 8, monosomy 7, del(7q),monosomy 5, del(5q), and del(20) may be detected in up to 50% of patients. Prognosis depends on degree of cytopenia. The frequency of transformation to acute leukemia at two years is about 10%. The overall median survival is approximately 30 months.

References

WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition

International Agency for Research on Cancer (IARC), 2008.

REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS
REFRACTORY ANEMIA WITH MULTI-LINEAGE DYSPLASIA AND RINGED SIDEROBLASTS