Incidence of myelodysplastic syndromes in a Swedish population.

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.

Application of megakaryocytic morphology in diagnosing 5q- syndrome.

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.

Bone marrow karyotype and prognosis in primary myelodysplastic syndromes.

Bone marrow karyotype, survival time, and the rate of progression to leukaemia were studied in 111 unselected patients with primary myelodysplastic syndromes. The 49 patients (44%) with clonal chromosome aberrations had survival time (median 29 months) similar to that found in the 62 patients with normal bone marrow karyotype (24 months, p greater than 0.10). The presence of multiple (greater than 2) abnormalities (17 patients) was strongly associated with poor prognosis, with a median survival of only 7 months (p less than 0.001). Prognostic information could be attributed to 2 specific abnormalities, del(5q) and -7: Presence of del(5q) as the sole anomaly was associated with long survival (36+ months), whereas monosomy 7 was a bad prognostic sign (6 months). The risk for leukaemia development correlated neither with the number of chromosome abnormalities nor with any particular anomaly. Our findings demonstrate the prognostic importance of quantifying the complexity of bone marrow chromosome changes. They also emphasize that different specific abnormalities convey widely different prognostic information in primary myelodysplastic syndromes.

Classification of patients with myelodysplastic syndromes according to the FAB co-operative group's proposals. Proposals for a redefinition of blast cell type II.

53 patients with the myelodysplastic syndromes (MDS) were classified according to the proposals of the FAB cooperative group 1982. 29 patients had refractory anaemia (RA), 10 refractory anaemia with excess of blasts ( RAEB ), 5 RAEB in transformation, 7 RA with ringed sideroblasts and 2 chronic myelomonocytic leukaemia ( CMML ). Counting blast cells type I and II involved no difficulties. 4 of 15 patients who developed acute myeloid leukaemia (AML) according to the FAB classification of 1976 did not fulfill the new 1982 criteria for AML. A redefinition of the blast cell type II to include a more granulated blast cells, without the characteristics of promyelocytes, would solve this problem. We conclude that a redefinition of the blast cell type II might turn out to be useful.

Prediction of survival in adults with acquired Bi- or pancytopenia.

In a retrospective study of 44 adults with acquired bi- or pancytopenia without evidence of any causal disorder, the survival curve suggested the existence of a subgroup of short survivors, mainly with aplastic anaemia, with death within 4 months. The initial values of 14 single clinical, blood and bone marrow variables were significantly associated with survival less than 4 months. Stepwise multiple logistic regression analyses identified 2 combinations of variables displaying significant simultaneous associations with short survival: (i) increased % of non-myeloid bone marrow cells and haemorrhagic manifestations initially; (ii) increased % of non-myeloid marrow cells, circulating erythroblasts and no history of any drug exposure. The predictive capacities of a resulting estimate of the probability of short survival and of previously introduced prognostic indices were approximately equal. The frequency of a correct prediction of a survival shorter than 4 months was in the range of 0.71-0.78 and that of longer survival in the range of 0.74-0.94.

The liver in Hodgkin's disease--I. Clinico-pathological relations.

A retrospective analysis of staging results from 308 patients with Hodgkin's disease (HD) was performed in order to relate clinico-pathological findings with respect to the liver to other staging results and to prognosis. Thirty-four patients had clinically enlarged liver, 80 had increased serum-enzyme levels indicating possible liver damage, but only 10 patients had biopsy-proven histologic evidence of HD in the liver (7 primary biopsies, 3 re-biopsies). Among the prognostic correlations not only advanced stage and liver infiltrates were connected to poor prognosis, but also--even in early stages--elevated serum enzyme values (S-GOT and alkaline phosphate).

The liver in Hodgkin's disease--II. Histopathologic findings.

Histopathologic findings in 459 liver biopsies performed in 308 patients with Hodgkin's disease were reviewed. Typical Hodgkin infiltrates were found in only 11/459 biopsies (2.4%) and infiltrates suspect for Hodgkin's disease in only 8/459 (1.7%), whereas unspecific changes were more common. Lymphocytic infiltration was seen in 47% of the biopsies and focal necrosis in 19%. No correlation could be demonstrated between unspecific changes and clinical characteristics, including prognosis.

The applicability of simple blood and bone marrow variables in prediction of acute leukaemia in patients with acquired bi- or pancytopenia.

A retrospective study of 44 patients presenting with acquired bi- or pancytopenia (B/PC) and no evidence of any causal disorder was carried out. 12 patients developed acute non-lymphoblastic leukaemia after 2-51 months. Compared to the nonleukaemic subjects the preleukaemic patients at the onset of B/PC had significantly higher counts of myeloblasts and other myelopoietic cells in the bone marrow, more cellular marrows due to more dense cellularities of all compartments, higher blood granulocyte and monocyte counts, more pronounced anisocytosis, and in some cases a small number of circulating myeloblasts. A stepwise multiple logistic regression analysis identified 3 combinations of variables displaying significant simultaneous association with subsequent leukaemic transformation. Among these the combination of marrow myeloblast percentage and blood granulocyte count might be applicable to early prospective identification of patients with preleukaemic B/PC.