ABSTRACT
The myelodysplastic syndromes (MDS) have attracted great interest during the last decade. Unfortunately there is not much material published concerning the incidence of these conditions. We present epidemiologic data on MDS based on case registration of patients in a well-defined population and as a comparison similar data on acute myeloid leukaemia (AML). Between the years 1978-1992 we registered 120 cases of MDS and 146 cases of AML. The median age for all the MDS cases was 74.1 years for men and 78.2 years for women. Among haematologists there is a suspicion that the incidence of MDS is rising. Our study does not support this opinion. We have divided the study period into 5-year periods and the crude incidence has been 3.2, 4.1 and 3.5/100,000/year for each period. In the age group over 70 years MDS was more frequent than AML and in the last 5-year period the incidence was 15.0/100,000/year for MDS compared to 10.2/100,000/year for AML. In conclusion MDS is quite common among elderly people and there is no evidence for a rising incidence during the last 15 years.
Subject(s)
Myelodysplastic Syndromes/epidemiology , Acute Disease/epidemiology , Adult , Aged , Aged, 80 and over , Female , Humans , Incidence , Leukemia, Myeloid/epidemiology , Male , Middle Aged , Myelodysplastic Syndromes/chemically induced , Sweden/epidemiologyABSTRACT
In order to evaluate the diagnostic importance of the megakaryocytic morphology in the 5q- syndrome we studied the bone marrow from 48 unselected patients with myelodysplastic syndromes (MDS). 44 cases were primary and 4 secondary to cytostatic drug treatment or irradiation. There were 24 cases with chromosome anomalies, of whom 10 had del (5q). 4 of these had refractory anaemia (RA) with 5q- as the sole anomaly (group A), 2 had RA with 5q- and additional chromosome anomalies consisting of trisomy 8 (group B); 3 patients had RA with excess of blasts (RAEB) and complex, karyotypic changes also including 5q- (group B). Changes of the same type were found in 1 case of multiple myeloma with secondary MDS. All 6 RA patients with 5q- had characteristic megakaryocytes. More than 50% of the cells had no more than 2 nuclear segments, and predominantly had a diameter of 30 micron or more. No other patient with RA showed this picture. Only 1 patient with RAEB 5q- in group B had the same megakaryocytic changes. We conclude that diagnosis of a 5q- syndrome may be strongly suspected in cases of RA with these bone marrow changes. In cases of RAEB 5q- group B the bone marrow examination did not reveal the same consistent changes.
Subject(s)
Chromosome Aberrations , Chromosomes, Human, Pair 5/ultrastructure , Megakaryocytes/pathology , Myelodysplastic Syndromes/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Anemia, Refractory/genetics , Anemia, Refractory/pathology , Anemia, Refractory, with Excess of Blasts/genetics , Anemia, Refractory, with Excess of Blasts/pathology , Cell Nucleus/pathology , Female , Humans , Male , Middle Aged , Myelodysplastic Syndromes/classification , Myelodysplastic Syndromes/genetics , Myelodysplastic Syndromes/pathologyABSTRACT
A 62-year-old male patient with refractory anemia with excess of blasts had a t(3;8)(q26;q24) as an acquired chromosomal abnormality in the bone marrow. This aberration has not been previously reported as a sole anomaly in any type of neoplasm, including myelodysplastic or myeloproliferative disorders. The findings of disturbed thrombocytopoiesis with high platelet counts and micromegakaryocytes in the bone marrow support the idea that alteration of the transferrin receptor gene, localized to 3q26, may be of pathogenetic significance.
