Two Cases of Primary Acquired Sideroblastic Anemia: Special References to the Bone Marrow Culture Findings

Abstract

Bone marrow culture was performed in two cases of primary acquired sideroblastic anemia (PASA) to examine whether stem cell abnormalities existed or not, and it's clinical usefulness to predict the prognosis of the disease was evaluated.
The diagnosis of PASA was made on the basis of the following findings: (1) erythroid hyperplasia with ringed sideroblasts in marrow. (2) anemia refractory to Vit. B₆ administration. (3) absence of the history of other diseases and drugs which were known to be associated with sideroblastic anemia.
The first patient was a 69-year-old man with severe ancmia. He required frequent blood transfusions and died of heart failure probably caused by hemochromatosis eight months after admission. Another was a 66-year-old woman with mild anemia and she maintained the same hemoglobin level without blood transfusion.
The bone marrow cultures revealed the decrease not only in CFU-E but also in CFU-C. CTU-E and CFU-C were much decreased in severe anemic case than in mild anemic case. Ringed sideroblasts were shown by the iron staining technique among erythroblasts in CFU-E culture and were found not in colonies but in clusters. All the colonies were consisted of non-sideroblastic cells.
These findings suggest that PASA is one of the disorders involved with pluripotent hemopoietic stem cells and that ringed sideroblasts in PASA are derived from abnormal clone which has not potency to form colonies. Furthermore it seemed that the level of CFU-E growth is helpful to predict the prognosis of PASA.