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

The classification of acute leukemia has almost invariably been based on the morphologic diagnosis into two broad categories: acute lymphocytic and acute myeloid leukemia. Despite the wide range of morphologic variation in both groups, strict criteria to define the subgroups have only recently been proposed. The conventional markers for B and T cells are now being applied to leukemic cells as are cytochemistry and electron microscopy, terminal deoxynucleotidyl transferase, serum lysozyme, and surface markers, E-rosettes, membrane immunoglobulin, antinull acute lymphocytic leukemia antiserum, and Fc and C3 receptors. The myelodysplastic syndromes may mimic acute leukemia and it is important that they be identified and treated appropriately. The high incidence with which chronic myelomonocytic leukemia terminates in acute leukemia suggests that it is a preleukemic condition, whereas refractory anemia with excess blasts and acquired idiopathic sideroblastic anemia may have long, drawn-out courses. Only a small population of patients with the latter conditions develop acute leukemia.
 ...
PMID:Classification of acute leukemia. 33 70

The authors consider the clinical and biological data of Acquired Idiopathic Sideroblastic Anemia (AISA). The physiopathology of the syndrome is discussed; the relationships between pathologic sideroblastosis, dyserythropoiesis and ferrokinetic modifications are pointed out. The associated abnormalities of granulocytic and megacaryocytic series linked AISA to other myelodysplasia.
 ...
PMID:[Acquired idiopathic sideroblastic anemia (author's transl)]. 38 22

Detailed clinical and cytogenetic studies were performed in five patients who had abnormal hematopoiesis and an acquired deletion of an F-group chromosome. Cytogenetic analyses, with banding techniques, of cells from bone marrow, spleen, or unstimulated peripheral blood showed a partial deletion of the long arm of one chromosome 20 [del(20)(q11)] in all five patients. Three patients had myeloproliferative disorders of uncertain classification, the fourth had possible preleukemia, and the fifth had acute myelomonocytic leukemia. Although the five cases showed certain similarities, the clinical and hematologic findings seen with the 20q- abnormality were not specific. None of the patients showed evidence of polycythemia vera or idiopathic acquired refractory sideroblastic anemia, two diseases previously associated with the 20q-. Our studies indicate that the 20q- abnormality is not limited to diseases primarily affecting erythropoiesis but that it can be found in the broader spectrum of myeloid disorders. In polycythemia vera, the 20q- has sometimes been regarded as a possible result of previous therapy with cytotoxic agents; however, four of our patients were untreated when the deletion was first noted.
 ...
PMID:Deletion of the long arm of chromosome 20 [del(20)(q11)] in myeloid disorders. 69 93

In an attempt to identify prognostic factors for survival and leukemic transformation, 235 untreated patients with primary myelodysplastic syndromes (MDS) were analyzed in a single center retrospective study. To the well known FAB classification of MDS a supplementary group of patients with pure sideroblastic anemia (PSA) was added, characterized by the absence of dysplastic features of non-erythroid cells. Accordingly, the morphological subtypes were refractory anemia (RA), n = 55; PSA, n = 40; RA with ring sideroblasts (RARS), n = 33; RA with excess of blasts (RAEB), n = 53; RAEB in transformation (RAEB/T) n = 29; and chronic myelomonocytic leukemia (CMML), n = 25. Having screened 28 clinical, cytological, and laboratory parameters by univariate analysis, multiple regression analysis identified six variables with independent prognostic value: percentage of bone marrow blasts, serum LDH activity, PSA, hemoglobin concentration, age, and platelet count. If patients with PSA were excluded, the FAB classification no longer contributed independent prognostic information. Based on the results of this multivariate analysis, a simple scoring system was devised for predicting the survival of patients with MDS. A score of unity was allocated to each of the following parameters: bone marrow blasts greater than or equal to 5%, LDH greater than 200 U/I, hemoglobin less than or equal to 9 g/dl, and platelets less than or equal to 100 x 10(9)/I. As a function of their total score, patients were divided into three risk groups (group A, score 0; group B, score 1-2; group C, score 3-4), which differed significantly in both survival and rates of leukemic transformation. The cumulative survival 2 years after diagnosis was 91% in group A, 52% in group B, and 9% in group C (p less than 0.00005). The actuarial risk of transformation to acute myeloid leukemia at 2 years was 0, 19, and 54%, respectively (p less than 0.05). The inclusion of LDH enzyme levels qualified this scoring system for an accurate assessment of patients with CMML whose prognosis is viewed too favorably when rated by other scores. Furthermore, this score was able to identify those patients with RA and RARS who, without showing an excess of marrow blasts, have an unfavorable prognosis.
 ...
PMID:Primary myelodysplastic syndromes: analysis of prognostic factors in 235 patients and proposals for an improved scoring system. 173 14

The median age of occurrence of primary acquired sideroblastic anemia is 74.4 years, with a range of 12 to 96 years (calculated from 452 cases). Of 445 cases, 60.4% were male, and 39.6% female. Not only primary acquired sideroblastic anemia, but the entire myelodysplastic syndrome must be considered as a disease of old age.
 ...
PMID:Primary acquired sideroblastic anemia and myelodysplastic syndrome from a geriatric point of view. 187 85

The authors made radioisotope examinations of the erythrokinetics in 21 patients with primary myelodysplastic syndrome: refractory anaemia and sideroblastic anaemia. He investigated the importance of changes of several conventional erythrokinetic parameters as well as the turnover of transferrin molecules which is an indicator of erythropoietic activity independent on sideraemia. The author presents ferrokinetic and erythrokinetic findings associated with myelodysplastic syndrome, as described in the literature. Sideroblastic anaemia produces a typical picture during examination of the erythrokinetics, quite different from other sub-types of the disease in refractory anaemia the changes of parameters are very variable.
 ...
PMID:[Erythrocyte kinetics in myelodysplastic syndromes]. 202 Oct 77

Myelodysplastic syndromes (SMD) were studied in 58 patients (37 men, 21 women; mean age 61 years, range 18-81) who were grouped according to FAB criteria (Table 1). None of them showed a secondary SMD to medullary toxic agents or cytostatic treatments although 5 presented concomitant neoplastic disease. Morphologic alterations in peripheral blood smears and bone marrow were registered by 3 hematologists working independently. The intracellular and extracellular iron deposits were evaluated in every case with Perls; peroxidase activity was determined in 16 patients and intraleucocitary alkaline phosphatase reaction was carried out in 17 patients. Twenty five patients (43%) had refractory anemia (RA); 10 (17%) sideroblastic anemia; 13 (25%) refractory anemia with excess of blasts (AREB); 3 (5%) AREB in transformation (AREB-T) and 7 myelomonocytic leukemia (LMMC). Clinical manifestations at diagnosis are described in Table 2. In the observation period there were cases of anemia requiring transfusion, bacterial infections, muco-cutaneous hemorrhage and hemorrhagic episodes in the central nervous system. In the bone marrow smears the cellularity was normal or increased in 53 cases and diminished in only 3. The degree of dysplastic characteristics (erythroid, granulocytic and megakaryocytic) ranged from low to severe. It was low in most of AR, being the erythroid population the most affected in AS and the granulocytic one in AREB and AREB-T. Patients with LMMC showed similar characteristics to those with myeloproliferative syndromes and the differential diagnosis were sometimes difficult, accounting for their separate inclusion in Table 4. Out of 23 patients, 5 presented clonal pathology detected in cytogenetic studies.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Myelodysplastic syndrome: experience of the Study and Treatment of Bone Marrow Failure Group]. 213 Feb 4

In order to detect possible relationships between cytogenetic abnormalities and morphologic features in myelodysplastic syndromes (MDS), 48 patients with MDS were investigated. Clonal cytogenetic abnormalities were present in bone marrow cells from 27 patients (56%). The most frequent single anomaly was del (5 q) (10 cases), followed by monosomy 7 (3 cases), trisomy 8 (3 cases) and del (20 q) (2 cases). Complex anomalies were present in 6 patients. Morphologically, according to the French-American-British (FAB) classification: 17 cases were considered as refractory anemia (RA), 17 as RA with excess of blasts (RAEB), 2 as RAEB in transformation, 2 as acquired idiopathic sideroblastic anemia and 10 as chronic myelomonocytic leukemia. With regard to the FAB classification, del (5 q) was often associated with RA and complex cytogenetic anomalies with RAEB. When myelodysplasia was studied in individual myeloid lineages, del (5 q) was associated with hypolobulated megakaryocytes, monosomy 7 with micromegakaryocytes and complex chromosomal anomalies with the association of two or more features of dysmegakaryocytopoiesis. Del (11 q) was associated with increased iron storage and del (20 q) with marked dyserythropoiesis. No correlation was observed between cytogenetic anomalies and features of dysgranulocytopoiesis.
 ...
PMID:Correlations between cytogenetics and morphology in myelodysplastic syndromes. 233 81

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.
 ...
PMID:[Myelodysplastic syndromes in the aged]. 236 31

Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) was examined in vitro in 23 patients with aplastic anemia and 14 with myelodysplastic syndrome (MDS) to investigate the clinical use of rh-Ep for these diseases. Bone marrow mononuclear cells were cultured by methylcellulose methods for CFU-E and BFU-E assays. In normals, the CFU-E numbers reached a plateau of increase at Ep doses of almost 2-5 units, and no further increase was observed with the addition of larger Ep doses. In aplastic anemia, the responses of CFU-E to Ep were relatively good in nonsevere type and generally poor in severe type. However, the CFU-E numbers increased with increasing doses of Ep in some of the patients with aplastic anemia. Among the patients with MDS, the responses of CFU-E to Ep were relatively good in primary acquired refractory anemia (PARA) and primary acquired sideroblastic anemia. On the other hand, the responses of CFU-E to Ep were poor in refractory anemia with an excess of blasts (RAEB) and RAEB in transformation among the MDS patients. BFU-E responses to Ep were poor in severe aplastic anemia, RAEB, and RAEB-T. However, there are Ep responsive patients in some of aplastic anemia and PARA. High titers of rh-Ep were suggested to be effective clinically in some patients with aplastic anemia and those with PARA.
 ...
PMID:Responsiveness of bone marrow erythropoietic stem cells (CFU-E and BFU-E) to recombinant human erythropoietin (rh-Ep) in vitro in aplastic anemia and myelodysplastic syndrome. 238 70


1 2 3 4 5 6 7 8 Next >>