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)

In a retrospective study of 352 patients with primary myelodysplastic syndromes, 61 (17.3%) revealed myelofibrosis in bone marrow biopsies. The fibrosis was observed to occur mostly focally (41/61 cases), and collagen deposits were found very rarely (4/61). The histopathology of bone marrow biopsies revealed hyperplasia and disturbed differentiation in megakaryopoiesis; the frequency and grade of dysplasia in megakaryopoiesis increased with advancing myelofibrosis. Reticulin fibrosis occurred in all subtypes of MDS; however, there was a higher incidence in chronic myelo-monocytic leukaemia (CMMoL). The frequency of cytogenetic aberrations was significantly higher in the MDS cases with myelofibrosis, compared to the cases without fibrosis. Clinical data showed significantly lower values of haemoglobin and lower platelet counts in MDS with myelofibrosis. Life expectancy was reduced to 9.6 months, compared with 17.4 months in MDS without fibrosis. In refractory anaemia, the survival times were 10.0 months in MDS with myelofibrosis, compared to 28.9 months in MDS without myelofibrosis. 36.6% of the patients with MDS and myelofibrosis developed a transformation into ANLL during the course of the disease. Myelofibrosis therefore seems to herald a poor prognosis.
 ...
PMID:Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients. 159 1

Three groups of patients with leukaemia and myelodysplasia were assessed with regard to the blood product support they required during their period of bone marrow hypoplasia following treatment. One group received myelo-ablative remission-induction chemotherapy followed by appropriate consolidation therapy (two courses in patients with acute myeloid leukaemia and one or two intensification courses in patients with acute lymphoblastic leukaemia); whilst the other two had 'conditioning' with chemotherapy and radiotherapy prior to autologous bone marrow transplantation (auto-BMT) or T cell depleted allogeneic bone marrow transplantation (allo-BMT). There was no statistically significant difference in blood product requirements between the three groups. However, platelet requirements during remission-induction chemotherapy alone were significantly less than for allo-BMT or auto-BMT. Platelet requirements for patients undergoing auto-BMT were also significantly higher than for patients receiving consolidation chemotherapy; and were required for a longer period than for patients receiving allogeneic-BMT. There was no difference in blood product support between ABO matched and mismatched transplants within the allogeneic group, but the presence of graft versus host disease and/or cytomegalovirus infection did significantly increase the requirements for blood product support.
 ...
PMID:Blood product support in patients undergoing chemotherapy and autologous or allogeneic bone marrow transplantation for haematological malignancies. 193 21

Seven cases of myelodysplastic syndrome with myelofibrosis, which is defined using the following criteria: (1) pancytopenia with less than 5% blasts in the peripheral blood; (2) minimal or no splenomegaly; (3) myelofibrosis with cellular marrow; (4) absence of diffuse proliferation of blasts in the bone marrow; and (5) presence of myelodysplastic features of bone marrow or peripheral blood cells, are presented. They were in the range of 52-82 years old and consisted of 3 males and 4 females. Six out of 7 cases developed into acute leukaemia after 5 to 8 months from the onset and died from between 2 weeks to 8 months from the evolution to leukaemia. The type of leukaemia was acute myeloblastic in 3 patients, and acute myelo-megakaryoblastic in 3 patients. Another patient died of severe hepatic injury after 5 months from the onset of the disease. These findings revealed that the complication of myelofibrosis in the patients with myelodysplastic syndrome was an indicative sign of rapid progression to overt leukaemia or otherwise poor prognosis for survival. In addition myelodysplastic syndrome is thought to be major primary disorder for acute myelofibrosis. Myelodysplastic syndrome with myelofibrosis is closely associated with the neoplastic proliferation of megakaryoblasts in a considerable number of patients.
 ...
PMID:Myelodysplastic syndrome with myelofibrosis: myelodysplastic syndrome as a major primary disorder for acute myelofibrosis. 206 Feb 60

Analysis of myelodysplastic syndromes (MDS) in 77 patients terminating in "overt" leukemia revealed sequential changes of marrow morphology with respect to cellularity and involvement of lineages, indicating that different "forms" of MDS represent in reality different phases. Three main phases were observed, which occurred in the following non-reversible sequence: (a) hypocellular dysplasia (H), (b) hypercellular dysplasia with predominance of erythropoiesis (E), and (c) with predominance of myelo(mono)poiesis (MM). Published studies suggest that these phases represent different manifestation stages of the stem cell lesion leading to MDS. Manifestation may probably be promoted by factors causing marrow aplasia. Transition to "overt" leukemia in several cases occurred locally, not throughout the whole marrow. In some patients a mature myelo(mono)-cytic cell population showed the capacity for widespread tissue infiltration, characteristic of leukemic cells. This argues against the theory that "overt" leukemia is the result of continuous dedifferentiation in MDS, but favours the concept of multiple initiating "events" leading to evolution of a leukemic subclone within a myelodysplastic clone. A connection between leukemia differentiation and MDS phase, from which leukemia developed, was also observed indicating that the risk of different stem cell subpopulations to become the target of the leukemia initiating "event" varies during the course of myelodysplasia.
 ...
PMID:Preleukemic myelodysplastic syndromes (MDS): pathogenetical considerations based on retrospective clinicomorphological sequential studies. 258 93

PMN elastase is a useful additional parameter in the differential diagnosis of the leukaemias. In all patients with myelocytic leukaemias there were elevated levels of elastase-alpha 1-proteinase inhibitor (E-alpha 1PI), while in the lymphatic leukaemias complexed elastase levels were decreased. The highest values were found in the peripheral blood plasma and bone marrow plasma of patients with CML. Despite high E-alpha 1PI concentrations there were no signs of bleeding or consumption of plasmatic coagulation factors. In AML a wide range of E-alpha 1PI levels was observed, extending from slightly elevated to four hundred-fold increased. In myeloblastic leukaemias without maturation (FAB M 1) the concentrations of complexed elastase remained below 150 ng/ml. In myeloblastic leukaemias with maturation (FAB M2) the E-alpha 1PI values ranged between 214 ng/ml and 850 ng/ml (means = 402 +/- 69), and in myelo-monoblastic leukaemias (FAB M4) between 450 ng/ml and 720 ng/ml (means = 663 +/- 72). The only case of promyelocytic leukaemia (FAB M 3) exhibited an extremely high value of 4,550 ng/ml, while a monocytic leukaemia (FAB M5) showed an extremely low value of 5 ng/ml. During cytostatic therapy there was a rapid decrease in levels of complexed elastase, with E-alpha 1PI values returning to normal in remission. In recidivating cases there was an increase of E-alpha 1PI levels in AML and a decrease in ALL. There was a correlation between the E-alpha 1PI concentrations in peripheral plasma and leukaemic bone marrow infiltration, so providing a good basis for monitoring remission from leukaemia and indicating relapse. It was also interesting to observe an extremely low E-alpha 1PI level (5 ng/ml) in patients with myelodysplasia. Under Decortin/Plenastril therapy the concentration rose to 50 ng/ml. An E-alpha 1PI level of 10 ng per ml was observed in one case of Ranitidine agranulocytosis. Under corticoid therapy the value returned to normal within eight days.
 ...
PMID:The importance of granulocyte elastase in haematological diagnosis. 316 79

Bone marrow biopsies of patients with a myelodysplastic syndrome (MDS) may, in the absence of an increased number of blasts in the bone marrow smears, contain small clusters of immature precursors. The presence of these cell nests, previously described as "abnormal localized immature precursors" or ALIP, bears a strong prognostic value predisposing patients to early death with an increased risk to develop myeloid leukaemia. In order to describe and delineate this histological characteristic more precisely, we compared bone biopsies of patients with MDS, used in these previous studies, with bone marrow biopsies performed for staging procedures in patients with Hodgkin's disease, non-Hodgkin's lymphoma and carcinoma. From this comparison we conclude that immature precursors are readily differentiated from proerythroblasts, myeloblasts and small-sized megakaryocytes, and that they most probably represent precursors of the myelo-monocytic-erythroid series. A clearcut increase in their number, mostly resulting in the formation of small clusters, is only found in biopsies from patients with MDS.
 ...
PMID:The histological characterization of ALIP in the myelodysplastic syndromes. 376 79

The retrospective evaluation of bone marrow biopsies of 569 patients with primary myelodysplastic syndrome--pMDS--revealed 256 refractory anemias--RA--, 52 refractory anemias with ringed sideroblasts--RARS--, 133 refractory anemias with excess of blasts--RAEB--, 52 refractory anemias with excess of blasts in transformation--RAEB-t--, and 53 chronic myelo-monocytic leukemias--CMMOL--according to FAB-criteria, 23 patients were not otherwise specified (myelodysplastic syndrome: not otherwise specified--MDS.NOS--). RARS-patients had the best prognosis (median survival 41.9 months, incidence of leukemia 3.8%), followed by RA-patients (26.5 months, 16.4%), MDS.NOS-patients (22.4 months, 21.7%), CMMOL-patients (12.5 months, 49.1%). RAEB- and RAEB-t-patients had the worst prognosis (median survival time 8.5 and 4.6 months, incidence of leukemia 42.1% and 57.7%, respectively). But the survival times showed a considerable range in each FAB-subgroup with 0-154 months in RA or 0-52 months in CMMOL. To forecast life expectancy more precisely, a scoring system was developed using nine histopathological parameters, among which the three most important ones were determined: quantity of myeloblasts, myelofibrosis and ALIP's. The scoring system allows a determination of three risk groups with significantly different survival times. It is valid also for patients without increase of myeloblasts (< 5% myeloblasts in the bone marrow) and identifies high-risk MDS patients in this group. By this proposed scoring system, a prognostic approval in primary MDS can be achieved applying histopathology without regarding further methods herewith presenting a system which could be considered independently from hematologic, cytological or laboratory data.
 ...
PMID:Life expectancy in primary myelodysplastic syndromes: a prognostic score based upon histopathology from bone marrow biopsies of 569 patients. 781 8

The molecular determination of the monoclonal or non-clonal nature of polycythemia cases is now possible in all or almost all females by study of the methylation pattern of several polymorphic X-linked loci. However, the techniques used are sophisticated and costly and the results do not clarify all cases. Karyotypic studies of PV, despite the number of cases reported thus far, remain insufficient. The natural history of cytogenetic events and their mechanisms of occurrence are still poorly known. Unsuccessful examinations are around 10-15%. The proportion of patients with clonal anomalies, 10-15% at diagnosis, increases with time, myelo-ablative treatments and evolution to myelofibrosis/myeloid metaplasia, so as to be close to 100% in cases with myelodysplasia/acute transformation. A few of the most frequently found non-random anomalies among which in particular trisomy 8 and 9, double trisomy 8+9, 20q- and trisomy of part or totality of 1q have some degree of specificity. Other recurring aberrations, such as 13q- mainly occur in late stages of PV, in which complex, unstable karyotypes are found. Finally none of those anomalies can be considered as a primary lesion. The frequency of clonal evolution is a matter of discussion. Despite their theoretical interest, PV cytogenetic results are of little diagnostic value, and as regards prognosis, they are statistically significant, but are of poor value in most individual patients. In conclusion, it is felt that clonality and karyotype studies should be pursued in PV, completed by "interphase cytogenetics" and molecular biology investigations, with theoretical aims rather than for immediate practical reasons.
 ...
PMID:Clonality and karyotype studies in polycythemia vera. 803 35

Myelosarcoma (granulocytic sarcoma) is a rare tumor seen in patients with known hematologic malignancies such as leukemia, myelodysplastic syndromes and myeloproliferative disorders, as well as in non-leukemic patients. A correct diagnosis in these cases is often difficult, and these are more commonly misdiagnosed as large cell lymphoma. We describe two women, 40 and 89 years of age respectively, in whom a myelosarcoma involving the neck lymph nodes was diagnosed by cytological examination of a fine needle aspiration biopsy, one and 10 weeks before the onset of acute myelogenous leukemia. The fine needle aspiration technique allowed a quick and unexpected diagnosis to be made demonstrating granulocytic differentiation and the presence of myelo-monocytic cells within lymphatic tissue. The simplicity of the procedure, coupled with its reliability and rapidity suggest that fine needle aspiration biopsies should be used more widely as a first choice method in the diagnostic evaluation of palpable lymph nodes.
 ...
PMID:Myelosarcoma preceding acute leukemia diagnosed by fine needle lymph node aspiration: report of two cases. 822 Jan 39

Recently an increase of the elderly patients with hematological malignancies has been pointed out. We analyzed second malignancies in elderly patients with hematological malignancies (95 age 65 or more), and made a comparative study with non-elderly case for the past 5 years. Second malignancies were observed in 26 cases out of the total of 282 hematological malignancies (9.2%). The percentage of patients with second malignancies in the elderly group (19/95; 20%) was significantly higher than that of the non-elderly group (7/187; 3.7%). Among the all kinds of hematological malignancies, the second malignancies were mainly observed in cases with myelodysplastic syndrome and chronic myelo-proliferative disorder. Colon carcinoma, gastric carcinoma and lung carcinoma accounted for nearly half of all the second malignancies. On 11 of the 26 cases with second malignancies, the first malignancies had been treated with some anti-cancer drug such as alkylating agents. Development of a second cancer was greater in cases in which the first hematological malignancy was treated with alkylating agents more than in cases in which the first carcinoma was not treated with alkylating agents.
 ...
PMID:[Elderly cases of hematological malignancies with second malignancies]. 823 Jul 94


1 2 3 Next >>