Posted on February 17, 2010.
What is refractory anemia with excess blasts? can someone please clarify this for me? Thank you? Name of disease;
Myelodysplastic syndromes
Synonyms;
anemia refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory, with excess blasts in transformation, chronic myelomonocytic leukemia
Description;
The myelodysplastic syndromes (MDS) is a group of diseases in which the production of blood cells by the bone marrow is disrupted. Unlike leukemia, in which a specific type of blood cells (WBC) is produced in too large numbers, the production of any and sometimes all types of blood cells is affected.
The myelodysplastic syndromes were previously covered by many names, including preleukaemia. The term Preleukaemia no longer used because it is very misleading. Even if a minority of MDS patients develop acute leukemia, most do not. When leukemic transformation occurs, it is acute myeloid leukaemia1. This form of acute myeloid leukemia is generally more difficult to treat than primary acute myeloid leukemia (cases occurred in patients without disease of the bone marrow before).
The bone marrow in myelodysplastic syndrome is typically more active than normal and yet the number of blood cells in circulation are reduced. Because most cells produced in the bone marrow are defective and are destroyed before they leave the bone marrow into the blood. The characteristic of the myelodysplastic syndromes is the combination of hyperactive marrow with low blood cell counts. A reduction in the number of all types of blood cells is called pancytopenia.
There is a degree of overlap between MDS and aplastic anemia. It can sometimes be difficult to distinguish between aplastic anemia and a subtype of MDS in which the bone marrow is underactive. This form of MDS is called hypoproliferative or hypoplastic myelodysplastic syndrome. Fortunately, the discrimination between these conditions is not critical for treatment planning.
RAEB is a disease of the bone marrow (MDS) classified by the FAB (French-American-British) system that includes four types (1) refractory anemia (RA), (2) RA with ringed sideroblasts (RARS) (3) RA with excess blasts (RAEB, 5-20% marrow blasts) and (4) RAEB in transition to AML (RAEBT, 20-30% marrow blasts).
RAEB is basically a type of refractory anemia (ie, refractory to other treatments as blood transfusions) that results from the bone marrow produces too many cells, the myeloid component (that is, white blood cell). In RAEB, myeloid cells are often structurally abnormal.
Refractories means responding to treatment. With an excess of blasts I worry about the development of leukemia as in aplastic anemia.