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)

A survey of the results of marrow transplantation for severe aplastic anemia (SAA) and myelodysplastic syndrome (MDS) in Japan is reported. Of the 152 patients with SAA, 109 were alive between 2 and 132 months following transplantation and the probability of survival at 5 years was 70% (for the patients with grafts from HLA-matched siblings) and 100% (for the patients with grafts from monozygous twins). Survival rate at 3 years for the patients with grafts from family members other than HLA-matched siblings was 46%. The chance of survival was influenced by conditioning regimen and recipient's age. Recipients with sustained engraftment had a significantly higher survival rate than those with graft failure (83% vs 11%, p less than 0.001). Since 1985, the results of transplantation from HLA-matched siblings have improved and the 3-year survival is more than 90% for patients under 20 years old. For MDS, the actuarial survival at 3 years was 42%. The chance of survival was not influenced by the FAB classification, patient's age, patient's sex, interval from diagnosis to transplant, karyotype anomaly or graft versus host disease.
 ...
PMID:Bone marrow transplantation for patients with severe aplastic anemia and myelodysplastic syndrome. 262 63

Acquired chromosomal rearrangements in acute nonlymphocytic leukemia (ANLL) have been linked to specific clinicopathologic features that suggest new disease subtypes. In this collaborative study, we report five patients with ANLL and a t(3;5) in their leukemic cells. At diagnosis, four of the patients had a t(3;5) as their sole karyotypic anomaly; the remaining patient had additional structural and numerical abnormalities. Careful cytogenetic analysis indicated that the breakpoints of this rearrangement are 3q25.1 and 5q34, in contrast to the various breakpoints reported in earlier studies (3q21----3q25 and 5q31----5q35). The karyotypic, morphologic, and clinical characteristics of this group, as well as those of 14 previously reported patients with the t(3;5), were compared to identify any features that might warrant consideration of a specific syndrome. The available information indicates a worldwide distribution and a nearly equal male:female ratio for patients with this translocation. The median age of the group, 37 years, was younger than that of all patients with ANLL, 49 years. A preceding myelodysplastic syndrome was observed in three patients. The limited numbers of observations on leukocyte count, hemoglobin level, and platelet count precluded meaningful comparison with data for ANLL patients in general. Although each FAB morphologic subtype, except M3, occurred in patients with a t(3;5), the frequency of M6 was much greater than expected. Bone marrows from each of the five patients we report showed increased numbers of megakaryocytes; trilineage dysplasia was observed in the marrow of each of the four patients for whom it could be assessed. Taken together, these findings suggest that the t(3;5) may affect cells capable of differentiation into multiple lineages.
 ...
PMID:Clinicopathologic manifestations and breakpoints of the t(3;5) in patients with acute nonlymphocytic leukemia. 264 76

The concurrent occurrence of myelodysplastic syndrome and diabetes insipidus is very rare. In the available literature so far only three cases were described. In acute leukaemia the concurrence of the two diseases is rare. The authors describe two cases of concurrent diabetes insipidus and myelodysplastic syndrome with typical clinical and laboratory symptoms. A 27-year-old man with refractory anaemia (according to the FAB classification of myelodysplastic syndrome) died after 10 months from complications of acute leukaemia. In a 58-year-old female patients with refractory anaemia after two years remission the condition deteriorated and developed into RASEB (refractory anaemia with excess of blast cells) according to the FAB classification with partial remission of the disease after cytostatic treatment. During permanent substitution treatment (Adiuretin Spofa) diabetes insipidus was compensated in both diseases.
 ...
PMID:[Diabetes insipidus as the first cause of myelodysplastic syndrome]. 271 26

A male with myelodysplastic syndrome (MDS) following aplastic anemia is reported. The patient had been diagnosed as aplastic anemia at 8 years of age, and treated with blood transfusions, anabolic and glucocorticoid steroid hormones. Over a period of subsequent twelve years, he had remission and deterioration. At the age of 21, the patient developed a sudden progression of severe anemia, when his bone marrow showed hyperplasia with prominent dyshematopoiesis and excess of blasts, compatible with MDS by the definition of FAB classification. He received low dose Ara-C therapy, which was ineffective. Nine months later he developed acute monocytic leukemia (M5b) and died. Chromosomal analysis revealed 46, XY at the onset of aplastic anemia, 46, XY, del (6) (q21 q27) at the MDS and 46, XY, -7, +21, 6q-/47, XY, +Y, -7, +21, 6q- at the acute leukemic stage.
 ...
PMID:[An adolescent case of myelodysplastic syndrome following aplastic anemia]. 271 3

In vitro megakaryocytic colony formation by progenitors from the bone marrow was studied in 40 myelodysplastic syndrome patients. Megakaryocytic colonies were decreased in number or absent in 30 patients; only 10 showed normal colony growth. The growth correlated to some extent with the FAB-class. Patients with normal colony formation had either RA or RARS, but also in these two FAB-types half of the patients showed reduced megakaryocytic colony formation. Only 1 out of 15 patients with RAEB or RAEBt had normal megakaryocyte growth. The patients with CMML did not show any megakaryocytic colonies. The growth of erythroid colonies was normal in 3 patients and reduced or absent in the others. All 3 with normal erythroid colony formation also showed normal megakaryocyte growth, and all patients with normal megakaryocyte colony formation also had normal granulocyte-macrophage colony growth. Granulocyte-macrophage colony and cluster formation was normal in 17 patients. Defective formation of megakaryocytic, erythroid, and granulocyte-macrophage colonies was seen in 22 patients, compatible with a defect in a pluripotent stem cell. Megakaryocytic colony formation had no obvious correlation with any specific chromosome abnormality, and the distribution of the growth patterns was almost similar in patients with a normal and those with an abnormal karyotype.
 ...
PMID:Colony formation by megakaryocyte progenitors in myelodysplatic syndromes. 227 Oct 49

We report an autopsy case of myelodysplastic syndrome (MDS) in a 35-year-old male, who presented with pancytopenia and bleedings. Bone marrow specimens disclosed myelofibrosis and hypercellular marrow with more than 60% atypical erythroblasts in the bone marrow cells. Type I or type II blasts were less than 10% of the peripheral blood and bone marrow cells during the clinical course. At autopsy, infiltration by myeloid and erythroid cells and megakaryocytes was noted in the liver, spleen and lymph nodes. According to the FAB classification, this case might be classified into refractory anemia with excess of blasts (RAEB) or RAEB in transformation. However, the remarkable neoplastic proliferation of three haematopoietic cell lines also indicates acute myeloproliferative disorder such as acute myelofibrosis or acute panmyelosis.
 ...
PMID:An autopsy case of myelodysplastic syndrome (MDS): diagnostic problems between MDS and the other haematopoietic disorders including acute myelofibrosis. 274 51

Cytogenetic studies of bone marrow metaphases from a 17-year-old woman with acute myeloid leukemia revealed a leukemic clone characterized by the t(8;21)(q22;q22) characteristic of FAB-M2. The patient was treated and achieved transient remissions. On relapse, her leukemic clone had acquired, in addition to the t(8;21), the inv(16)(p13q22) characteristic of FAB-M4Eo and a 5q- of the type seen in various acute myeloid leukemias and myelodysplastic syndromes. This cell line persisted throughout the remainder of the patient's clinical course. There were no other clonal chromosome abnormalities observed. The observation of multiple chromosome mutations, usually regarded separately as primary, in a single leukemic clone is most unusual and raises questions about our concepts of the nature of primary acquired chromosome mutations in cancer.
 ...
PMID:Acquisition of additional primary chromosome abnormalities in the course of karyotype evolution in a case of FAB-M2 acute leukemia. 275 92

A multivariate analysis of clinical, biochemical and hematologic data was performed in 138 patients with myelodysplastic syndromes (MDS) in order to evaluate their prognostic significance. The most important individual variables, isolated in a previous univariate analysis, were placed in a multiple regression modeling procedure to identify major prognostic factors. Multivariate analysis tends to identify prognostic variables containing significant predictive information. Characteristics were examined on both continuous and binary bases. The FAB classification was the first parameter entered in regression equations of both models, followed by platelet count, systemic symptoms, bone marrow blast and infection. Our analysis confirms FAB classification as the best prognostic factor in MDS. It supports the previously predictive value of platelet count, hemoglobin level and bone marrow blast and recognizes the importance of systemic symptoms and infection as prognostic characteristics in MDS.
 ...
PMID:Prognostic factors in myelodysplasic syndromes: a multivariante analysis in 138 patients. 276 68

Cytogenetic studies detected abnormalities in 107 (43%) of the 247 patients in this series. Some degree of overt clinical progression occurred in 55 patients (22%), this being 29% of those patients with cytogenetic abnormalities and 17% of those with normal chromosomes. The presence and complexity of a clonal cytogenetic abnormality correlated with shorter survival. In each clone category of a complexity classification (simple, complex, very complex), patients with some normal cells appeared to have better survival than those with none. In multiple regression analyses, the prognostic value of chromosomes was independent of (and second in importance to) the FAB type of myelodysplastic syndrome (MDS) whichever chromosome classification was used. Patients with refractory anemia (RA) had the lowest incidence of chromosome abnormalities and no cases were found to have only abnormal cells (AA). A greater proportion of patients with refractory anemia with an excess of blasts (RAEB) and RAEB in transformation (RAEB-t) had clonal abnormalities. Morphology alone is not at present able to distinguish between RA or refractory anemia with ringed sideroblasts and similar disorders that may not be MDS in the strict sense. Demonstration of a clonal cytogenetic abnormality remains a positive indication of the presence of the neoplastic nature of the disease.
 ...
PMID:Clinical-cytogenetic correlations in myelodysplasia (preleukemia). 276 40

Pretreatment bone marrow biopsies of 63 patients with severe aplastic anaemia (SAA) who were not transplanted and of whom 55 received ATG, were evaluated according to the amount and character of residual haematopoiesis ('genuine' aplasia/intermediate/hypoplastic myelodysplasia (MD], inflammatory infiltrate (Te Velde & Haak, 1977, grade I/II/III), and number of mast cells (normal or slightly increased/increased). Of 61 evaluable biopsies, 47 were 'genuine' aplastic, 11 intermediate and three hypoplastic MD. Inflammatory infiltrates were graded as III in 36/60 evaluable biopsies, as II in 21 and I in three. A moderate to marked increase of mast cells was seen in 19/61. Of grade III patients, 86% had a less than 90 d interval between diagnosis and administration of ATG, versus 50% of grade I/II patients (P less than 0.01). No other correlations with pretreatment characteristics were found. No significant prognostic value for survival or response to ATG of any of these three criteria has been identified. More patients with grade III inflammation tended to show adequate recovery at 4 and 6 months after ATG. Stem cell damage, not identifiable morphologically, and/or impairment of accessory cells might play a major role in eventual outcome of SAA patients. Thirty-five patients are currently alive, median 3.8 years (up to 12.4) after ATG. Follow-up bone marrow aspirates and biopsies of 32 patients were evaluable and none showed normal haematopoiesis. One patient revealed persistent aplasia. Of the remaining 31, haematopoiesis was decreased in 14 and increased in eight. All had dyserythropoiesis, 28 dysplastic myelopoiesis and in 16/29 with evaluable megakaryocytes, dysmegakaryopoiesis was found. Sixteen patients had normo- to hypercellular bone marrows with two dysplastic cell lines (consistent with myelodysplastic syndrome (MDS) according to the FAB-group). The prognostic impact of the dysplastic abnormalities found in these patients needs longer follow-up. Close observation is indicated in view of the previously recognized, albeit uncommon, evolution of SAA to MDS/acute non-lymphocytic leukaemia.
 ...
PMID:Bone marrow histopathology of patients with severe aplastic anaemia before treatment and at follow-up. 278 55


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>