Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 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.
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PMID:Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients. 159 1

One hundred and thirty-three cases of myelodysplastic syndromes studied during the last ten years were revised. Of them, 79 were males and 54 females, and their ages ranged between 15 and 91 years (median, 69 years). Five patients (3.7%) had secondary myelodysplasias. The haematological phenotype (FAB) of the cases was: RA, 41.3%; SRA, 24%; RAEB, 18%; RAEBT, 3.7%; CMML, 8.3%. Leucopenia/thrombocytopenia without initial anaemia was present in 4.5% of the cases. Abnormal karyotype was found in 54 patients (40.6%), MIKA in 41 cases and MAKA in 13 cases. The cytogenetic anomalies most commonly found were +8, 5q-, -7, 11q- and 13q-. Cytogenetic abnormalities were commonest amongst the RAEB (50%), and least frequent in CMML (18.2%). Thirty-one patients evolved into acute leukaemia (29 ANLL and 2 ALL). Such blastic changes were more frequent in RAEB (62.5%) and rarest in SRA (9.4%), and they appeared mostly in patients with complex karyotype (MAKA) (53.8%) as compared with those who had normal karyotype (17.7%). Short-lasting complete remission was achieved by 40% of the patients treated with conventional chemotherapy. The survival of the group as a whole (median 30 months) varied in accordance with the haematological phenotype: SRA, 81 months; RA, 65 months; CMML, 13 months; RAEB +/- T, 8 months. The finding of a MAKA karyotype significantly shortened the survival (4 months) with regard to MIKA (44 months) or normal karyotype (39 months). The following median survivals were attained after patients' staging (Bournemouth's criteria): stage A, 84 months; stage B, 22 months, and stage C, 5 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Myelodysplastic syndromes. Hematologic phenotypes, cytogenetic expression and clinical course in 133 cases (1979-1989)]. 227 38

A 12-year old boy was admitted to Saitama Children's Medical Center because of fever and epistaxis. He had leukocytosis (WBC 40,800/microliters, blast 75%), anemia, thrombocytopenia and high levels of serum LDH, lysozyme, Vitamin B12, and plasma histamine. Bone marrow aspiration revealed hypercellular marrow with 31.2% blasts, 15.2% eosinophils, and 14.2% basophils. Blasts had Auer rods and were positive for peroxidase and negative for alpha-naphthyl butyrate esterase and PAS stainings. Ia, CD13 (My7), and CD19 (B4) antigens were expressed on his leukemic cells. Chromosomal study showed 46, XY, t(7;8) (q35;q22), del(9) (q13q22). Southern blot analysis using immunoglobulin constant region (C) probes revealed germline patterns of C mu, C kappa, C lambda, and breakpoint cluster region. A diagnosis of acute myelomonocytic leukemia (AMMoL, M4) was made. He attained a complete remission with daunorubicin and cytarabine, and 6 months later he received bone marrow transplantation from HLA-identical sister. This case had the common breakpoint 8q22 with ANLL with t(8;21) (q22;q22), and was unique AMMoL with proliferation of eosinophils and basophils in bone marrow.
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PMID:[Acute myelomonocytic leukemia (M4) with CD19 antigen expression, eosinophilia and basophilia in bone marrow]. 247 65

The authors examined 117 consecutive patients with acute leukemia within a few days of diagnosis. Sixty-six patients had acute nonlymphocytic leukemia, and 51 had acute lymphocytic leukemia. Forty-two percent of the patients had abnormal ocular findings related to leukemia. The authors found an association between the presence of intraretinal hemorrhages and thrombocytopenia for all patients. In addition, patients with acute lymphocytic leukemia and intraretinal hemorrhages had lower hematocrit levels than did those without such hemorrhage. The presence of anemia was related to the findings of white-centered hemorrhages in patients with acute nonlymphocytic leukemia. The presence of cotton-wool spots was not associated with hematologic parameters. Intraretinal hemorrhages, white-centered hemorrhages, and cotton-wool spots were all found more frequently in adults than in children.
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PMID:Leukemic retinopathy. Relationship between fundus lesions and hematologic parameters at diagnosis. 274 81

pSV2 neomycin DNA was electroporated into exponentially growing WEHI-3B D+ cells, and clones containing integrated pSV2 neomycin DNA were selected by treatment with the antibiotic Geneticin. Southern analysis after Eco RI or Bam HI and Hind III digestion demonstrated that two clones, Y1 and Y2, stably incorporated a single copy of intact pSV2 neomycin DNA into different sites. Exposure of parental WEHI-3B D+ cells to retinoic acid, aclacinomycin A, or adriamycin resulted in their differentiation to mature granulocytic cells. Clone Y1 had growth kinetics and numbers of differentiated cells comparable to those of the parental line when exposed to these differentiation-inducing agents. Furthermore, this clone was leukemogenic in BALB/c mice. The disease presented as a moderate anemia, a peripheral blood granulocytosis, a shift in the differential to immature granulocytic forms, and a thrombocytopenia. Although there was no change in bone marrow cellularity, in situ hybridization to identify the presence of the neomycin resistance gene mRNA indicated infiltration of leukemia cells (up to 30%) into this tissue compartment. The observed hematological parameters were indicative of a late stage of disease resembling human acute nonlymphocytic leukemia. Clone Y1, therefore, would appear to be an in vivo model of acute nonlymphocytic leukemia that may be useful for the development of therapeutic strategies to more successfully treat these diseases. Because we have demonstrated that this line is capable of differentiation in vitro and can be stably carried in vivo, it would also be useful for the identification of therapeutic agents capable of initiating the differentiation of leukemia cells in situ.
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PMID:Development of neomycin-resistant WEHI-3B D+ murine cells as an in vivo model of acute nonlymphocytic leukemia. 291 37

A 5-year-old girl with refractory anaemia with excess of blasts in transformation (RAEBT) terminating in acute nonlymphocytic leukemia is reported. The patient's serum had an inhibitory effect on normal hemopoiesis. T cell subsets defined by monoclonal antibodies revealed a decrease in helper/inducer T cell population (OKT4-positive cells) and inversion of the T4/T8 ratio. It is suggested that imbalance in immunoregulatory T cells, which has a crucial role in the control of hemopoiesis, may be a primary event in the pathogenesis of myelodysplastic syndrome which eventually develops into an overt leukemia.
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PMID:Disturbances of T lymphocyte subsets preceding refractory anemia with excess blasts in transformation in a child. 312 67

The clinical, hematologic, and phenotypic features of 28 patients with acute leukemia with megakaryocytic involvement (AMKL) were analyzed. The prevalence of this type of leukemia in the entire series was 11.6%, with a higher incidence among patients with acute transformation of a previous myeloproliferative disorder (MPD) (24%) than among the transformed myelodysplastic syndrome (13%) patients. The incidence in the "de novo" ANLL was 8% and 16% among secondary leukemias. The presence of bone marrow fibrosis together with low WBC and normal or increased platelet counts despite a severe anemia are the most relevant features in these patients who otherwise displayed an apparently poor prognosis. Megakaryoblasts were morphologically recognized more frequently in the acute transformations of MPD than in de novo ANLL. Only two cases were considered pure AMKL, and in the remaining 26 patients, megakaryoblasts coexisted with other granulomonocytic and/or erythroid populations. Antiglycoprotein IIIa (anti-GPIIIa) (C17) and anti-GPIIb/IIIa (CDw41-, J15-) antibodies are probably the best markers for AMKL, although the monoclonal antibody against GPIX (FMC25) was also positive in a majority of cases but in a lower percentage of cells. On the other hand, megakaryoblasts were generally negative for granulocytic or monocytic markers (CD13, CD14, CD15); the expression of HLA-DR antigens in these cells was variable. Our present results indicate that megakaryoblastic involvement is more common than previously recognized. This is true not only in acute transformed leukemias but also in de novo ANLL. Although the diagnosis of these cases should be based on megakaryocytic markers, it is often possible to suspect a diagnosis according to certain clinical and hematologic features.
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PMID:Leukemias with megakaryoblastic involvement: clinical, hematologic, and immunologic characteristics. 316 92

We analyzed the natural history of idiopathic refractory sideroblastic anemia (IRSA) in 37 patients studied between 1969 and 1986. Although erythroid abnormalities were prominent in all, 12 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines, and nonrandom chromosomal aberrations were observed in five of 23 patients studied for such defects. Measurements of erythroid marrow function showed in most cases erythroid expansion with ineffective erythropoiesis. In seven patients, however, the erythroid activity was found to be inappropriately low for the degree of anemia. Transfusion dependence occurred in 26 of 37 cases. Iron overload was a common feature at presentation but produced clinical manifestations of hemochromatosis only in those patients who subsequently had a regular need for blood transfusions. Five patients progressed to bone marrow failure, and another five patients (two of whom had monosomy 7) evolved into acute nonlymphocytic leukemia (ANLL). The median survival was 72 months, with a high transfusion requirement, multilineage defects, and inappropriately low erythroid proliferation being associated with a poor prognosis. The most common causes of death were complications of iron overload and evolution into ANLL. We conclude that (a) the natural history of IRSA is characterized by an initial phase of erythroid hyperplasia and ineffective erythropoiesis, which is usually stable for many years but in a subset of patients may be followed by a phase of marrow failure with or without the later emergence of leukemic blasts; (b) peripheral blood counts, measurement of erythroid marrow function, and chromosomal analysis are useful for identifying subjects at risk of evolution into marrow failure or ANLL; and (c) IRSA patients with no need for blood transfusions are very likely to be long survivors, whereas those who become transfusion dependent are at risk of death from the complications of secondary hemochromatosis.
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PMID:Natural history of idiopathic refractory sideroblastic anemia. 333 99

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.
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PMID:Refractory anaemia according to the FAB classification: a report on 69 cases. 336 22

The evolution of leukaemia was studied prospectively in 29 patients with myelodysplastic syndrome (MDS) followed for 2-6 years by sequential blast counts, cell kinetics derived from quantitative 14C-autoradiography and karyotype analysis. Overt leukaemia developed in seven patients. Two distinct patterns of leukaemic evolution were identified. The first was characterized by a gradual increase in blast cell count and in the frequency of labelled blasts, and a corresponding reduction in myeloid maturation index indicating increased intracompartmental myeloblast divisions and premature myeloid cell death. A second pattern of leukaemic evolution was marked by a sudden rise in the blast cell population in a previously stable MDS. This rise was attributed both to an increased rate of blast proliferation, and the accumulation of non-proliferating blasts. In an additional patient with smouldering ANLL and multiple karyotype abnormalities, transient clinical remission took place following prednisone and oxymetholone therapy, characterized by a sideroblastic morphology, normal karyotype, and persistence of a highly abnormal myeloid maturation index. The sudden emergence of overt leukaemia in previously stable MDS in some of our patients and the temporary reversal of overt leukaemia into sideroblastic anaemia in one case, lend support to the notion of leukaemic evolution by several steps of transformation. On the other hand, the gradual transition of MDS into overt leukaemia in other patients is compatible with a single step leukaemia transformation, although the possibility of clonal disease prior to the development of MDS cannot be excluded with certainty.
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PMID:Myelodysplastic syndromes: evolution of overt leukaemia by one or several steps of transformation. 367 2


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