Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventy-five cases of myelodysplastic syndromes (MDS) in the aged (over 60 years) were analysed for hematological findings, immunological parameters and response to treatment in respect to prognostic significance. They were diagnosed according to the FAB classification, but patients with hypoplastic marrow were included if myelodysplasia was evident. Thirty-four percent of patients with primary acquired refractory anemia (PARA) or primary acquired sideroblastic anemia (PASA), and 36% of patients with RA with excess of blasts (RAEB) had hypoplastic bone marrow. The positive rates of antinuclear antibody in PARA or PASA, and of rheumatoid factor in PARA or PASA and in RAEB were higher than those in normal aged controls. Cellularity of bone marrow was inversely related to the length of survival. Among the patients with PARA or PASA, survival time was significantly longer in the group of hypoplastic bone marrow than in the group of hyper- or normo-plastic bone marrow, and in the group of good responder to treatment than in the group of poor responder and nontreated patients.
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PMID:[Myelodysplastic syndromes in the aged]. 236 31

A case of Philadelphia (Ph1) chromosome positive acute myelogeneous leukemia (AML) following a refractory anemia with excess of blasts (RAEB) with 8 trisomy is reported. The 80-year-old man developed pancytopenia during the course of follow-up after the surgical operation of the carcinoma of the sigmoid colon and the rectum for which no irradiation therapy nor chemotherapy had been applied. The diagnosis of RAEB was made according to the diagnostic criteria proposed by FAB co-operative group. Chromosomal analysis revealed 8 trisomy in 54% of the metaphases of bone marrow cells. The remainders showed normal karyotype without Ph1 chromosome. He was on androgenic steroid and activated Vitamin D3 without significant changes in the clinical and the hematological features until 3 months later when many atypical blasts appeared in the peripheral blood. The diagnosis of AML (M2) was made. Chromosomal analysis revealed Ph1 chromosome with the typical 9;22 translocation in 100% of the examined cells. 8 trisomy was not detected any more. Southern blot analysis using bcr probe showed bcr rearrangement. He was treated with a small doses of Ara-C. There was some reduction in the number of blasts in the peripheral blood. However, he died of septicemia 2 months later. The present case indicates that Ph1 positive acute leukemia with bcr rearrangement is not necessarily considered as a blastic transformation of chronic myelogeneous leukemia and such a cytogenic abnormality can appear in a leukemic transformation of myelodysplastic syndrome.
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PMID:[Acquisition of Philadelphia chromosome with bcr rearrangement concomitant with transformation of refractory anemia with excess of blasts with 8 trisomy into acute myelogenous leukemia]. 236 38

Ten characteristics of bone marrow (BM) biopsies in paraffin sections, obtained at diagnosis from patients with myelodysplastic syndromes (MDS) classified according to the FAB criteria, were analysed to identify both the most relevant morphologic data and any possible influence on survival. Agreement between two observers was obtained for 94% of the data. BM cellularity was increased in 63% of the cases and was higher in refractory anaemia with excess of blasts (RAEB). RAEB in transformation (RAEB-t) and chronic myelomonocytic leukaemia (CMML) (P = 0.001). Dysmegakaryopoiesis and dyserythropoiesis were present respectively in 83% and 72% of the cases, with slight differences among the FAB subtypes. Abnormal localization of immature precursors (ALIP) was found in more than half of the cases and somewhat more frequently seen in the RAEB + RAEB-t + CMML group (P = 0.07). Eosinophilia, plasmacytosis and reticulin fibrosis were evident in 26%, 18% and 47% of the cases respectively. Cellularity (P = 0.006), eosinophilia (P = 0.009) and, to some extent, dysmegakaryopoiesis (P = 0.07) bore a certain relationship with survival on univariate analysis. The presence of ALIP was not seen to affect the outcome. Multivariate analysis showed that the cellularity and presence of dysmegakaryopoiesis, in BM biopsy, added significant independent prognostic information to that achieved with age, platelet count and proportion of blast cells in BM aspirate, three variables with proven prognostic value in MDS patients. Using a regression model including these five characteristics we have stratified the patients into low, intermediate and high-risk groups with different survivals (P = 0.00001). The present findings show that BM biopsy is able to provide both morphological characteristics and information about the prognosis of survival, and should thus be included in the initial evaluation of MDS.
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PMID:Bone marrow biopsy in myelodysplastic syndromes: morphological characteristics and contribution to the study of prognostic factors. 237 20

Clinical features, as well as morphology, immunophenotype and cytogenetics were retrospectively studied in 20 patients with an original diagnosis of erythroleukaemia (EL) reclassified according to the FAB criteria. Fifteen patients had de novo EL, five patients had therapy-related EL. Myelodysplasia preceded the onset of EL in eight cases and myelodysplastic features involving multiple haemopoietic lineages were observed at leukaemia presentation in all cases. Immunologic findings confirmed multilineage involvement, showing sub-population of cells expressing platelet-associated markers in more than 50% of cases tested and the presence of a myelomonocytic component, besides glycophorin A-positive cells. Cytogenetically, major karyotype aberrations (MAKA), defined by the presence of three or more aberrant events in the same clone, were observed in 14 cases, minor karyotype aberrations (MIKA) were observed in four cases and normal karyotype in two cases. No differences in the cytological-cytogenetic picture of our patients with de novo EL, and with therapy-related EL were found suggesting that aetiological factors and/or pathogenetic mechanisms common to EL and secondary leukaemia may exist. All patients with MAKA had leftward shift of erythropoiesis with proerythroblasts and basophilic erythroblasts usually representing more than 50% of all erythroid cells. In patients with MIKA or normal karyotype, maturatio of erythroid cells, though morphologically abnormal, was quantitatively preserved and early erythroblasts never exceeded 25% of erythroid cells. Clinically, the haemoglobin level at presentation, as well as in the proportion of patients achieving complete remission after chemotherapy, appeared to be lower in the maturation arrest-MAKA group as compared to the preserved maturation-MIKA/normal karyotype group. Median survival was shorter in the former group (3.5 months) than in the latter (median 13 months). Morphologic-immunologic-cytogenetic studies thus allow for the identification of two distinct cytogenetic-clinicopathological types of EL.
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PMID:Morphologic, immunologic and cytogenetic studies in erythroleukaemia: evidence for multilineage involvement and identification of two distinct cytogenetic-clinicopathological types. 238 68

Cytogenetic studies of 91 consecutive patients with therapy-related myelodysplasia or overt acute nonlymphocytic leukemia disclosed characteristic defects of chromosome 7 in 48 cases and of chromosome 5 in 21 cases. The chromosome 5 abnormalities were consistently present in all abnormal mitoses at the time of diagnosis, as were the chromosome 7 abnormalities in 45 of the 48 patients. Various abnormalities, primarily of the short arm of chromosome 17, were observed in 13 cases, abnormalities of the long arm of chromosome 21 were observed in 12 cases, and rearrangements of 11q23 were seen in nine cases. Thirteen patients presented a normal karyotype. Previous therapy with alkylating agents, the presence of an initial myelodysplastic phase, and abnormalities of chromosome 7 or 5 were interdependent. Patients with 11q23 rearrangement typically developed overt leukemia of FAB types M4 or M5a without myelodysplasia and with a short latent period. Evaluated by Cox regression analysis, complete remission of the primary malignancy and a malignant lymphoma as primary tumor were the two most important and independent prognostic factors indicating a longer survival (P = .008). In addition, the platelet count at diagnosis was a significant prognostic factor (P = .01). For the subgroup of 62 patients with myelodysplasia, the number of chromosome aberrations, the percentage of blasts in the bone marrow, and the hemoglobin level were other significant and independent prognostic factors (P = .05, .05, and .004, respectively). The most important predictive factor for a favorable response to intensive antileukemic chemotherapy in overt leukemia was the absence of a preceding myelodysplastic phase (P = .0014).
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PMID:Chromosome aberrations and prognostic factors in therapy-related myelodysplasia and acute nonlymphocytic leukemia. 240 Aug 4

We are reporting four patients who presented with a persistent increase of mature neutrophils and band forms, no monocytosis in repeated examinations and with dysplastic features in blood and bone marrow. Two of them developed acute myeloid leukemia (AML; FAB classification M1) and the other two were characterised by a progressively aggravating myelodysplasia. All the patients showed a poor response to treatment and died within a period of time ranging from 14 to 80 months after diagnosis. We are proposing the term chronic neutrophilic leukemia with dysplastic features (CNL-D) for this entity, and we believe that it represents a variant of the myelodysplastic syndromes.
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PMID:Chronic neutrophilic leukemia with dysplastic features. A new variant of the myelodysplastic syndromes. 212 Aug 84

Superoxide (O2-) production by neutrophils was examined in 21 patients with myelodysplastic syndrome (MDS), including 13 with primary MDS (p-MDS) and 8 with therapy-related MDS (t-MDS). O2-production of MDS patients was significantly less than that of healthy controls (2.81 +/- 3.51 vs 6.19 +/- 2.41 nmol/min/10(6) PNMs, p less than 0.005). Although no relationship between pathogenesis (primary or therapy related), FAB type and levels of O2- production was observed, O2- production of 11 patients with abnormal bone marrow karyotypes was significantly reduced than that of 10 other patients with normal karyotypes. Furthermore, five t-MDS patients with monosomy for all or part of chromosome no. 7 (-7 or 7q-) showed the lowest level of O2- production, which was significantly different from the level in the patients with other abnormal karyotypes (0.61 +/- 0.29 vs 1.80 +/- 0.31, p less than 0.001). These results suggest that the long arm of chromosome no. 7 may in part play an important role for O2- production by neutrophils.
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PMID:[Impaired superoxide production by neutrophils in myelodysplastic syndrome: correlation with bone marrow karyotype]. 255 93

Systematic clinical and laboratory observations of 64 cases of myelodysplastic syndrome (MDS) were made. The results showed that malignant clone already exists in the bone marrow of patients with MDS. Eighteen cases of them have transformed into AML. The rates of transforming into AML in RAEB, RAEB-T and CMML were markedly higher than that in RA. The courses of MDS which transformed into AML from the various types were different. The transformation in RA was obviously longer than those in RAEB and RAEB-T. Two cases transformed into myelofibrosis. Diagnosis of CMML according to the FAB classification criteria revealed that it has two forms. One shows only increase of mature monocytes and it has no relation with the types of acute leukemia to be transformed. It is considered as a reactive monocytosis. The other form, in addition to increase of mature monocytes, also has increase of a few monoblasts and promonocytes. These are considered as true CMML and usually quickly developed into M4 or M5 which are related with monocytes.
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PMID:[A clinical study on myelodysplastic syndrome. Report of 64 cases]. 259 Dec 61

Clinical features, prognosis and factors influencing survival, as well as long-term evolution of the disease were analyzed in 130 patients with myelodysplastic syndromes (MDS) with particular reference to the refractory anemia with excess of blasts (RAEB). Survival of patients with 3 FAB subtypes, RAEB, RAEB in transformation (RAEB-T) and chronic myelomonocytic leukemia (CMMoL) showing excess blasts was uniformly poor relative to primary acquired refractory anemia (PARA) and primary acquired sideroblastic anemia (PASA). The degree of cytopenias, karyotypic abnormalities, bone marrow cellularity, transition to acute leukemia were not reliable prognostic parameters for discrimination of RAEB with poor or good prognosis. Disease transition was frequently observed in our MDS patient population, at an overall incidence of 37.7%. Transition of PARA to RAEB occurred after a prolonged course in some patients.
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PMID:Clinical aspects of the myelodysplastic syndromes (MDS) with special reference to refractory anemia with excess of blasts (RAEB). 261 56

The paper presented a case of a patient with type III by FAB myelodysplastic syndrome, in whom during a low dose cytarabine therapy ventricular fibrillation occurred. Authors discussed roles of anemia, ischemic heart disease and hypokalemia as factors which could increase circulatory system sensitivity on cytarabine action.
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PMID:[Ventricular fibrillation in a patient with myelodysplastic syndrome treated with small doses of cytarabine]. 262 13


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