22 results on '"Anemia, Refractory pathology"'
Search Results
2. Differences in the distribution of subtypes according to the WHO classification 2008 between Japanese and German patients with refractory anemia according to the FAB classification in myelodysplastic syndromes.
- Author
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Matsuda A, Germing U, Jinnai I, Araseki K, Kuendgen A, Strupp C, Iwanaga M, Miyazaki Y, Hata T, Bessho M, Gattermann N, and Tomonaga M
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Anemia, Refractory pathology, Asian People, Female, Germany, Humans, Male, Middle Aged, Myelodysplastic Syndromes pathology, Prognosis, Retrospective Studies, World Health Organization, Young Adult, Anemia, Refractory classification, Anemia, Refractory ethnology, Myelodysplastic Syndromes classification, Myelodysplastic Syndromes ethnology
- Abstract
We reported the different clinical features between Japanese and German refractory anemia (RA) patients in FAB classification. We re-analyzed the clinical features by WHO classification revised in 2008. The frequencies of refractory cytopenia with unilineage dysplasia (RCUD) and myelodysplastic syndrome-unclassified (MDS-U) with pancytopenia in Japanese patients were higher than in German patients (p<0.001). Refractory cytopenia with multilineage dysplasia patients showed the most unfavorable prognosis in both countries. The higher frequencies of MDS-U with pancytopenia and RCUD in Japanese patients may influence the different clinical characteristics between Japanese and German FAB-RA patients., (Copyright (c) 2010 Elsevier Ltd. All rights reserved.)
- Published
- 2010
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3. Spectrum of mutations in RARS-T patients includes TET2 and ASXL1 mutations.
- Author
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Szpurka H, Jankowska AM, Makishima H, Bodo J, Bejanyan N, Hsi ED, Sekeres MA, and Maciejewski JP
- Subjects
- Aged, Aged, 80 and over, Anemia, Refractory metabolism, Anemia, Refractory pathology, Anemia, Sideroblastic metabolism, Anemia, Sideroblastic pathology, Dioxygenases, Humans, Immunoenzyme Techniques, Middle Aged, Polymerase Chain Reaction, Polymorphism, Single Nucleotide genetics, Thrombocytosis metabolism, Thrombocytosis pathology, Anemia, Refractory genetics, Anemia, Sideroblastic genetics, DNA-Binding Proteins genetics, Mutation genetics, Proto-Oncogene Proteins genetics, Repressor Proteins genetics, Thrombocytosis genetics
- Abstract
While a majority of patients with refractory anemia with ring sideroblasts and thrombocytosis harbor JAK2V617F and rarely MPLW515L, JAK2/MPL-negative cases constitute a diagnostic problem. 23 RARS-T cases were investigated applying immunohistochemical phospho-STAT5, sequencing and SNP-A-based karyotyping. Based on the association of TET2/ASXL1 mutations with MDS/MPN we studied molecular pattern of these genes. Two patients harbored ASXL1 and another 2 TET2 mutations. Phospho-STAT5 activation was present in one mutated TET2 and ASXL1 case. JAK2V617F/MPLW515L mutations were absent in TET2/ASXL1 mutants, indicating that similar clinical phenotype can be produced by various MPN-associated mutations and that additional unifying lesions may be present in RARS-T., (Copyright (c) 2010 Elsevier Ltd. All rights reserved.)
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- 2010
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4. The significance of bone marrow cell morphology and its correlation with cytogenetic features in the diagnosis of MDS-RA patients.
- Author
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Liu D, Chen Z, Xue Y, Lu D, Zhou Y, Gong J, Wu W, Liang J, Ma Q, Pan J, Wu Y, Wang Y, Zhang J, and Shen J
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Diagnosis, Differential, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Retrospective Studies, Anemia, Refractory pathology, Bone Marrow Cells pathology, Chromosome Aberrations, Myelodysplastic Syndromes pathology
- Abstract
Besides cytopenia, dysplasia is crucial characteristic of MDS-RA. To summarize the morphological features that contribute to the diagnosis of MDS-RA, 48 RA patients with abnormal karyotype were analyzed for the features of morphological and cytogenetical abnormalities and the relationships between them. 46 MDS-RA patients with normal karyotype and 207 patients with non-MDS anemia were enrolled into control groups. More conspicuous and diverse dysplasia can be found in abnormal karyotype MDS-RA than those in control groups (P<0.05). Apparent dysplasia in granulocyte and megakaryocytoid lineages may provide valuable evidence for the diagnosis. Dysplasia occurred more frequently in patients with severe chromosome abnormalities.
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- 2009
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5. Myelodysplasia or myelodysplastic syndrome?
- Author
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Giagounidis AA
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Chromosome Aberrations, Diagnosis, Differential, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Retrospective Studies, Anemia, Refractory pathology, Bone Marrow Cells pathology, Myelodysplastic Syndromes pathology
- Published
- 2009
- Full Text
- View/download PDF
6. Presence of peripheral blasts in refractory anemia and refractory cytopenia with multilineage dysplasia predicts an unfavourable outcome.
- Author
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Knipp S, Strupp C, Gattermann N, Hildebrandt B, Schapira M, Giagounidis A, Aul C, Haas R, and Germing U
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Anemia, Refractory genetics, Anemia, Refractory, with Excess of Blasts genetics, Anemia, Refractory, with Excess of Blasts mortality, Anemia, Sideroblastic pathology, Chromosome Aberrations, Female, Humans, Male, Middle Aged, Myelodysplastic Syndromes genetics, Pancytopenia mortality, Prognosis, Risk, Survival Rate, Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts pathology, Myelodysplastic Syndromes mortality
- Abstract
The World Health Organization (WHO) assigns myelodysplastic syndrome (MDS) to RA/RCMD/RARS/RSCM/5q- syndrome, if medullary blasts are <5% and peripheral blast (PB) count < or =1%. In 1103 patients with these diagnoses, we analysed survival and risk of AML evolution depending on the presence of PB. Median survival in the group with 1% PB (n=74) was significantly lower as compared to those without PB (20 versus 47 months, p<0.00005). Cumulative risk of AML was significantly higher in patients showing PB (p<0.00005). Median survival of patients with PB was not different from that of RAEB I. We therefore propose to consider patients with PB, regardless of medullary blast, as RAEB I.
- Published
- 2008
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7. The presence of clonal cell subpopulations in peripheral blood and bone marrow of patients with refractory cytopenia with multilineage dysplasia but not in patients with refractory anemia may reflect a multistep pathogenesis of myelodysplasia.
- Author
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Cermák J, Belicková M, Krejcová H, Michalová K, Zilovcová S, Zemanová Z, Brezinová J, and Sieglová Z
- Subjects
- Anemia, Refractory blood, Anemia, Refractory classification, Anemia, Refractory genetics, Chromosome Banding, Female, Humans, In Situ Hybridization, Fluorescence, Myelodysplastic Syndromes blood, Myelodysplastic Syndromes genetics, Reference Values, Telomere ultrastructure, Anemia, Refractory pathology, Bone Marrow pathology, Myelodysplastic Syndromes classification, Myelodysplastic Syndromes pathology
- Abstract
A clonal origin of hematopoiesis was studied by investigation of X-chromosome inactivation patterns (XCIP) in isolated granulocyte, CD14(+) and CD3(+) subpopulations obtained from bone marrow and peripheral blood of 36 female patients with primary myelodysplastic syndrome (MDS). Clonality was assessed by PCR amplification of polymorphic short tandem repeats of the human androgen receptor (HUMARA) gene and by investigation of silent polymorphism of iduronate sulphatase (IDS) or p55 genes. On the basis of results in a control group of 20 healthy age related females, a ratio of at least 9:1 between the two alleles was considered a significant marker of monoclonal hematopoiesis. Ten of the 11 patients with advanced forms of MDS (RAEB, RAEB-T, CMML) had clonal granulocytes and CD14(+) cells in peripheral blood. In patients with early disease, only 2 out of 11 patients (18%) with RA or RARS, according to WHO classification, had clonal granulocytes and CD14(+) cells in peripheral blood and bone marrow and 2 other patients with 5q-syndrome exhibited extremely oligoclonal granulocyte subpopulation in bone marrow. In contrast, we found clonal granulocytes in 12 out of 14 patients (86%) with refractory cytopenia with multilineage dysplasia (RCMD) and 8 of them simultanously exhibited clonal CD14(+) cells. Estimated 3 years survival of patients with early disease and clonal cell subpopulations was 61% as compared with 88% in patients without clonal hematopoiesis. Karyotype abnormalities were detected in 11 of the 25 females with early disease. Clonal patterns were present in 7 out of 8 patients with abberations diagnosed by routine cytogenetics, nevertheless, FISH revealed 5q deletion in 3 patients without signs of clonality in XCIP assay. No correlation was found between the presence of clonal subpopulations and the degree of telomere shortening in early MDS. Despite some limitations, the measurement of XCIP remains a sensitive tool for diagnosis of the first transforming mutation in the clonal development of MDS especially when combined with FISH and when an age related group is used to establish an appropriate allele ratio to exclude constitutional or acquired skewing. The occurrence of clonal cell subpopulations in most of the RCMD patients in contrast to RA may reflect a proposed multistep pathogenesis of MDS with dysplastic changes limited to erythropoiesis in early step and with subsequent development of multilineage dysplasia. The results also support the usefulness of separation of RCMD from 'pure' RA; however, a more complex insight combining different molecular techniques performed in a large number of patients is needed for refined classification of MDS on the basis of new molecular prognostic factors and for indication of more effective targeted therapy.
- Published
- 2005
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8. Philadelphia chromosome positive myelodysplastic syndrome and acute myeloid leukemia-retrospective study and review of literature.
- Author
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Keung YK, Beaty M, Powell BL, Molnar I, Buss D, and Pettenati M
- Subjects
- Acute Disease, Aged, Anemia, Refractory pathology, Chromosomes, Human, Pair 22 genetics, Chromosomes, Human, Pair 9 genetics, DNA, Neoplasm genetics, Female, Humans, Leukemia, Myelogenous, Chronic, BCR-ABL Positive pathology, Leukemia, Myeloid pathology, Male, Middle Aged, Myelodysplastic Syndromes pathology, Retrospective Studies, Anemia, Refractory genetics, Leukemia, Myelogenous, Chronic, BCR-ABL Positive genetics, Leukemia, Myeloid genetics, Myelodysplastic Syndromes genetics, Translocation, Genetic
- Abstract
We conducted a retrospective study to define the significance of Philadelphia chromosome (Ph) in myelodysplastic syndrome and acute leukemia in the adults at this institution and the literature was reviewed. One hundred forty-eight cases of t(9;22)(q34;q11) were identified for the period September 1993 through August 2001. The presentation of 124 cases (84%) was that of typical CML in chronic phase. Nineteen cases (13%) presented as de novo ALL, two cases (1%) presented as de novo AML and three cases (2%) presented as myelodysplastic syndrome (MDS). The estimated incidences of t(9;22)(q34;q11) in ALL and AML are 21 and 0.6%, respectively. Ph+ AMLs are increasingly being reported with either M-BCR or m-BCR gene rearrangements, similar to those found with Ph+ ALL lending support to the notion that Ph+ AMLs are distinct entities and not merely blastic phases of undiagnosed CML. This is further supported by the existence of Ph+ MDS cases.
- Published
- 2004
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9. Philadelphia chromosome positive myelodysplastic syndrome and acute myelogenous leukemia.
- Author
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Advani AS
- Subjects
- Acute Disease, Anemia, Refractory genetics, Anemia, Refractory pathology, Chromosomes, Human, Pair 22 genetics, Chromosomes, Human, Pair 9 genetics, DNA, Neoplasm genetics, Humans, Leukemia, Myelogenous, Chronic, BCR-ABL Positive pathology, Leukemia, Myeloid pathology, Myelodysplastic Syndromes pathology, Translocation, Genetic, Leukemia, Myelogenous, Chronic, BCR-ABL Positive genetics, Leukemia, Myeloid genetics, Myelodysplastic Syndromes genetics
- Published
- 2004
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10. Classification of refractory anemia.
- Author
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Finn WG
- Subjects
- Anemia, Refractory pathology, Cell Lineage, Humans, Myelodysplastic Syndromes classification, Anemia, Refractory classification
- Published
- 2004
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11. Expression of TNF receptors and related signaling molecules in the bone marrow from patients with myelodysplastic syndromes.
- Author
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Sawanobori M, Yamaguchi S, Hasegawa M, Inoue M, Suzuki K, Kamiyama R, Hirokawa K, and Kitagawa M
- Subjects
- Adult, Aged, Aged, 80 and over, Anemia, Refractory metabolism, Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts metabolism, Anemia, Refractory, with Excess of Blasts pathology, Antigens, CD metabolism, Apoptosis drug effects, Bone Marrow Cells pathology, Down-Regulation, Female, Humans, Male, Middle Aged, Myelodysplastic Syndromes metabolism, Protein Serine-Threonine Kinases genetics, Protein Serine-Threonine Kinases metabolism, Proteins genetics, Proteins metabolism, RNA, Messenger metabolism, Receptor-Interacting Protein Serine-Threonine Kinases, Receptors, Tumor Necrosis Factor metabolism, Receptors, Tumor Necrosis Factor, Type I, Receptors, Tumor Necrosis Factor, Type II, Reverse Transcriptase Polymerase Chain Reaction, TNF Receptor-Associated Factor 1, TNF Receptor-Associated Factor 2, Tumor Necrosis Factor-alpha genetics, Tumor Necrosis Factor-alpha metabolism, Tumor Necrosis Factor-alpha pharmacology, Up-Regulation, fas Receptor genetics, fas Receptor metabolism, Antigens, CD genetics, Bone Marrow Cells metabolism, Gene Expression Regulation, Neoplastic, Myelodysplastic Syndromes genetics, Receptors, Tumor Necrosis Factor genetics, Signal Transduction
- Abstract
Myelodysplastic syndromes (MDS) are characterized by peripheral blood cytopenias despite hypercellularity of the bone marrow regarded as the result of ineffective hematopoiesis mainly caused by apoptosis. In this study, we examined the role of tumor necrosis factor (TNF)-induced apoptosis in the bone marrow cells of MDS patients. The constitutive expression of mRNA for TNF receptors (TNFR), including TNFRI and TNFRII, and the adapter molecules, such as the TNF receptor-associated death domain protein (TRADD), Fas-associated death domain protein (FADD), receptor interacting protein (RIP) and TNF receptor-associated factor 2 (TRAF-2) were analyzed by reverse transcriptase (RT)-PCR in bone marrow samples from control, MDS and AML cases. In bone marrow cells from refractory anemia (RA) patients, there was a significant increase in TNFRI expression as compared with control subjects. The expression of TNFRII was also up-regulated in RA cases. In contrast, RA with excess of blasts (RAEB), RAEB in transformation (RAEB-T) and AML cases revealed increased expression of TNFRII, whereas the expression of TNFRI was comparable to control subjects. Immunohistochemical staining revealed that the TNFRI, as well as TNFRII of MDS bone marrow was expressed mainly in hematopoietic cells, but not in macrophage-lineage stromal cells at the protein level. An increased constitutive expression of mRNA for TRADD, FADD and RIP and decreased expression of mRNA for TRAF-2 were observed in bone marrow cells from MDS patients, especially from RA patients, as compared with controls, although the differences were not significant. In many of the AML bone marrow samples, strong expression of TRAF-2 mRNA was marked, while expression levels of other proteins were similar to those in control subjects. These data suggested enhanced signaling by the TNFRI-TRADD-FADD pathway and suppressed signaling by the TRAF-2 pathway in RA. Thus, TNF-alpha-induced apoptosis may play a role in ineffective hematopoiesis in "early stage MDS" bone marrow, although the regulatory mechanisms for TNF-alpha-induced signaling would be complicated and not be simply explained only by these pathways.
- Published
- 2003
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12. Amifostine does not preferentially stimulate the growth of residual polyclonal progenitor cells in myelodysplastic syndromes.
- Author
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Delforge M, Raets V, Van Duppen V, and Boogaerts M
- Subjects
- Adult, Aged, Aged, 80 and over, Anemia, Refractory pathology, Antigens, CD34 metabolism, Cell Division drug effects, Cells, Cultured drug effects, Colony-Forming Units Assay, DNA Primers chemistry, Female, Granulocytes drug effects, Granulocytes metabolism, Granulocytes pathology, Hematopoietic Stem Cells metabolism, Hematopoietic Stem Cells pathology, Humans, Macrophages drug effects, Macrophages metabolism, Macrophages pathology, Middle Aged, Polymerase Chain Reaction, Receptors, Androgen metabolism, Amifostine pharmacology, Hematopoietic Stem Cells drug effects, Myelodysplastic Syndromes pathology, Radiation-Protective Agents pharmacology
- Abstract
Amifostine is a phosphorylated aminothiol that has besides anti-oxidative and cytoprotective properties, also survival- and growth-promoting effects on hematopoietic progenitor cells. Clinical studies have demonstrated that infusions with amifostine are able to increase erythro-, myelo-, and thrombopoiesis in some patients with myelodysplastic syndromes (MDS). Since clonal and non-clonal progenitors can coexist in early phase MDS, we have studied if amifostine exerts a selective growth-promoting effect on clonal or non-clonal cells. For this purpose, purified CD34(+) marrow progenitors from nine female MDS patients were grown in short- and long-term cultures. Clonality was studied on individual colonies using polymorphisms in the human androgen receptor assay (HUMARA) locus. Three patients had growth of residual non-clonal progenitors at baseline. Continuous exposure to 100nM amifostine exerted a growth-promoting effect on progenitors in 50% of the patients. HUMARA patterns of individual colony-forming unit granulocyte macrophage (CFU-GM; 5/9) and 5 week long-term culture-initiating cells (LTC-IC; 2/9) were compared without and with amifostine exposure. We did not observe preferential stimulation of clonal or non-clonal progenitors. Based on these results, the stimulation of committed and immature progenitor growth in MDS by amifostine, is non-selective and does not favor nor suppress the growth of residual non-clonal cells.
- Published
- 2003
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13. NOD/SCID mice transplanted with marrow from patients with myelodysplastic syndrome (MDS) show long-term propagation of normal but not clonal human precursors.
- Author
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Benito AI, Bryant E, Loken MR, Sale GE, Nash RA, John Gass M, and Deeg HJ
- Subjects
- Adult, Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts pathology, Animals, Antigens, CD analysis, Child, Clone Cells, Graft Survival, Humans, Immunophenotyping, Infant, Karyotyping, Mice, Mice, Inbred NOD, Mice, SCID, Middle Aged, Radiation Chimera, Receptors, Androgen genetics, Specific Pathogen-Free Organisms, Bone Marrow Transplantation, Hematopoietic Stem Cells cytology, Myelodysplastic Syndromes pathology, Transplantation, Heterologous
- Abstract
Sublethally irradiated NOD/SCID mice were transplanted with hematopoietic progenitor cells obtained from the marrow of patients with myelodysplastic syndromes (MDS). Engraftment of MDS cells, as determined by flow cytometry, was delayed compared to marrow from normal donors. Human CD38(+)CD34(-) cells were prominent in marrows and spleens of MDS chimeras. CD34(+)CD38(-), CD34(+)CD38(+) and T cells were also easily detected. Human myeloid cells (CD33(+); CD15(+)) were present in low proportions. No clonal precursors were identified by fluorescent in situ hybridization (FISH) or by molecular analysis of polymorphic X-linked markers in mice with documented engraftment of human cells more than 2 months after transplantation. These data indicate that human cells present in murine MDS chimeras, at the levels of sensitivity of our assays, were derived from residual normal cells in human MDS marrow, and suggest that the NOD/SCID environment was not conducive to the expansion of clonal MDS precursors. This model may allow identification of factors relevant for sustaining or expanding clonal precursors.
- Published
- 2003
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14. A prognostic impact of separation of refractory cytopenia with multilineage dysplasia and 5q- syndrome from refractory anemia in primary myelodysplastic syndrome.
- Author
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Cermák J, Michalová K, Brezinová J, and Zemanová Z
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Anemia, Refractory genetics, Anemia, Refractory pathology, Cell Lineage, Classification methods, Cytogenetic Analysis, Female, Humans, In Situ Hybridization, Fluorescence, Male, Middle Aged, Myelodysplastic Syndromes pathology, Prognosis, Survival Analysis, Syndrome, Treatment Outcome, Anemia, Refractory classification, Chromosome Deletion, Chromosomes, Human, Pair 5, Myelodysplastic Syndromes classification, Myelodysplastic Syndromes genetics
- Abstract
A prognostic impact of WHO classification of myelodysplastic syndrome (MDS) was studied in a group of 103 primary MDS patients with refractory anemia (RA) according to French-American-British (FAB) classification. Median survival of 37 patients with RA according to WHO criteria of 85.2 months was significantly different from that in both 37 patients with refractory cytopenia with multilineage dysplasia (RCMD) (47.0 months, P=0.002) and 29 patients with 5q- abnormality diagnosed by routine chromosome banding (36.2 months, P=0.0002). A more detailed karyotype analysis with fluorescent in situ hybridization (FISH) techniques confirmed 5q deletion as a sole cytogenetic abnormality in only 12 out of 29 patients, in 4 patients 5q- was associated with complex abnormalities involving 5q region, 13 patients had 5q deletion combined with further karyotype abberations outside 5q. No difference in median survival and estimated 3 years survival was observed between RA patients, patients with 5q- syndrome according to WHO morphology criteria and patients with 5q- as a single abnormality confirmed by FISH in contrast to patients with either additional 5q abberations or further karyotype changes not involving 5q. The same difference was also observed in time to 25% of patients evolving to acute myeloid leukemia (AML). Our study confirmed usefulness of separation of RCMD from RA. RCMD represents a poor prognostic subgroup of MDS clearly distinct from pure RA mainly due to short survival connected with progressive bone marrow failure and increased risk of leukemic transformation. We also suggest to define 5q- syndrome as primary MDS of FAB type RA with 5q deletion as a sole cytogenetic abnormality confirmed by FISH analysis. This definition enabled us to discriminate 5q- patients with favorable prognosis similar as in RA from those with poor outcome associated with 5q- combined with complex abnormalities involving either 5q or regions outside 5q.
- Published
- 2003
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15. Increased peripheral stem cell pool in MDS: an indication of disease progression?
- Author
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Vehmeyer K, Haase D, and Alves F
- Subjects
- Anemia, Refractory classification, Anemia, Refractory diagnosis, Anemia, Refractory pathology, Blood Cells pathology, Bone Marrow Cells pathology, Case-Control Studies, Colony-Forming Units Assay, Disease Progression, Hematopoietic Stem Cells physiology, Humans, Leukemia, Myelomonocytic, Chronic classification, Leukemia, Myelomonocytic, Chronic diagnosis, Leukemia, Myelomonocytic, Chronic pathology, Myelodysplastic Syndromes classification, Myelodysplastic Syndromes diagnosis, Myeloid Progenitor Cells pathology, Myelodysplastic Syndromes pathology
- Abstract
The colony-forming capacity of the peripheral blood stem/progenitor cells (PBSC) in different forms of myelodysplastic syndrome (MDS) was investigated. In most cases of refractory anemia (RA) the colony growth of PBSC was definitely reduced as compared to the controls. However, in RA with unfavorable chromosomal aberrations, in refractory anemia with ringed sideroblasts (RARS) and in advanced stages of MDS such as refractory anemia with excess blasts (RAEB) and refractory anemia in transformation (RAEB-t), the number of myeloid progenitor cells increased up to 100-fold. In chronic myelomonocytic leukemia (CMML), the increase was even more marked, up to 350-fold. Although the number of PBSC was strongly elevated, these cells were not able to restore hematopoiesis in vivo. In conclusion, the increase of circulating colony-forming cells (CFC) seems to be associated with disease progression, and thus, the evaluation of PBSC could be an important parameter in the diagnosis of MDS.
- Published
- 2001
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16. P53 overexpression in bone marrow biopsies in refractory anemia and aplastic anemia: impact of antibody selection.
- Author
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Elghetany MT
- Subjects
- Anemia, Aplastic genetics, Antibody Specificity, Biopsy, Humans, Immunohistochemistry methods, Reproducibility of Results, Retrospective Studies, Anemia, Aplastic pathology, Anemia, Refractory genetics, Anemia, Refractory pathology, Bone Marrow pathology, Genes, p53, Tumor Suppressor Protein p53 analysis
- Abstract
There is a growing interest in studying cell cycle and apoptosis in the myelodysplastic syndromes (MDS). p53 has been a major target of several studies. We examined the impact of antibody selection on p53 overexpression in bone marrow (BM) biopsies of 28 patients with refractory anemia (RA) in addition to 10 cases of aplastic anemia (AA) using three antibodies DO-7, PAb 1801, and PAb 240. DO-7 was positive in 68%, PAb 1801 in 18% and PAb240 in 4% of RA patients. All three antibodies were negative in AA. We conclude that antibody selection is an important variable in studying p53 in MDS regardless of the method of fixation or decalcification of BM trephine biopsies.
- Published
- 2000
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17. Mixed myelodysplastic and myeloproliferative syndromes.
- Author
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Neuwirtová R, Mociková K, Musilová J, Jelínek J, Havlícek F, Michalová K, and Adamkov M
- Subjects
- Adult, Aged, Aged, 80 and over, Anemia, Refractory complications, Anemia, Refractory genetics, Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts classification, Anemia, Refractory, with Excess of Blasts complications, Anemia, Refractory, with Excess of Blasts genetics, Anemia, Refractory, with Excess of Blasts pathology, Anemia, Sideroblastic complications, Anemia, Sideroblastic genetics, Anemia, Sideroblastic pathology, Female, Humans, Leukemia, Myelomonocytic, Chronic complications, Leukemia, Myelomonocytic, Chronic genetics, Leukemia, Myelomonocytic, Chronic pathology, Male, Middle Aged, Retrospective Studies, Thrombocytosis complications, Thrombocytosis pathology, Anemia, Refractory classification, Anemia, Sideroblastic classification, Leukemia, Myelomonocytic, Chronic classification, Thrombocytosis classification
- Abstract
Aplastic anemia, myelodysplastic syndromes (MDS) and chronic myeloproliferative diseases (MPD) are stem cell disorders. There is no clear-cut demarcation of them. Hypoplastic MDS displays features of aplastic anemia and MDS, on the other side mixed myelodysplastic and myeloproliferative syndromes (MDS-MPS) develop. In our collection of 566 MDS patients, features of myelodysplasia as well as myeloproliferation, MDS-MPS, were present in 25 patients (4.4%). Twelve patients had at the time of diagnosis megakaryocytic proliferation and thrombocythemia beside signs of MDS, and seven had myelodysplasia with granulocytic proliferation and leukocytosis. In another six patients, MDS was the first diagnosis and the proliferative phase developed later during the course of the disease. These patients can be characterized as MDS-MPS in evolution. All subjects had a variable degree of anemia. While the level of thrombocythemia has been relatively stable, the number of leukocytes has been progressive, but rarely extended beyond 100 x 10(9)/l. Ring-sideroblasts and myelofibrosis were frequent findings. Two more homogeneous MDS-MPS groups emerged in our analysis: sideroblastic anemia with thrombocythemia and a group fulfilling the criteria of Philadelphia chromosome negative and bcr-abl negative "atypical chronic myeloid leukemia (aCML)'. One patient with thrombocythemia and three with leukocytosis (23%) transformed to acute myeloid leukemia (AML). Men prevailed (12/13) in patients with leukocytosis and MDS-MPS in evolution. Of the 46% MDS-MPS patients with chromosomal aberrations, del(20)(q) is of interest.
- Published
- 1996
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18. Increased proliferation of eosinophil clusters in myelodysplastic syndromes.
- Author
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Koike M, Ishiyama T, Yokoyama A, Kawakami K, Nakamaki T, Tomoyasu S, and Tsuruoka N
- Subjects
- Aged, Anemia, Refractory pathology, Anemia, Refractory therapy, Cell Division, Colony-Forming Units Assay, Eosinophilia etiology, Eosinophilia pathology, Female, Granulocyte Colony-Stimulating Factor pharmacology, Granulocyte-Macrophage Colony-Stimulating Factor blood, Granulocyte-Macrophage Colony-Stimulating Factor pharmacology, Granulocyte-Macrophage Colony-Stimulating Factor therapeutic use, Hematopoietic Stem Cells pathology, Humans, Interleukin-3 blood, Interleukin-5 blood, Myelodysplastic Syndromes blood, Recombinant Proteins pharmacology, Recombinant Proteins therapeutic use, Bone Marrow pathology, Eosinophils pathology, Myelodysplastic Syndromes pathology
- Abstract
A patient with myelodysplastic syndromes (MDS) developed eosinophilia during treatment with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF). To study the mechanism of this eosinophilia, we investigated the proliferation of eosinophil colony-forming units (CFU-Eo) in nine patients and four healthy controls. Eosinophil clusters increased significantly in the patients (P < 0.01) compared with controls, but eosinophil colonies were not different between controls and MDS patients. In addition, the eosinophil clusters were significantly increased with rhGM-CSF in MDS patients compared with controls, although serum GM-CSF concentrations were similar in both groups. These results suggest that eosinophil clusters are increased in MDS either through abnormal progenitor proliferation or hypersensitivity to GM-CSF.
- Published
- 1995
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19. Clinical features of long-term survivors of refractory myelodysplastic anemias. A Japanese cooperative study.
- Author
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Yoshida Y, Oguma S, Uchino H, Maekawa T, and Nomura T
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Anemia, Refractory blood, Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts mortality, Bone Marrow pathology, Child, Child, Preschool, Female, Humans, Infant, Japan epidemiology, Male, Middle Aged, Prognosis, Retrospective Studies, Survival Rate, Anemia, Refractory mortality
- Abstract
Eighty-one of 473 patients with refractory myelodysplastic anemias registered by the Japanese Cooperative Study Group prior to January 1987 survived more than 5 years. At presentation, most patients had mild cytopenia, a less aggressive form of the disease (48 with refractory anemia, 23 with refractory anemia with ring sideroblasts, 10 with refractory anemia with excess of blasts), less blast cells in the marrow and blood, and onset at younger ages. Their clinical profiles 5 years after presentation showed no significant improvement. The results suggest that the long-term survivors were found in a subpopulation of patients with a favorable prognosis and that it is a part of the natural course rather than the results of treatment.
- Published
- 1992
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20. Heterogeneity of acquired idiopathic sideroblastic anaemia (AISA).
- Author
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Garand R, Gardais J, Bizet M, Bremond JL, Accard F, Callat MP, de Bouchony ET, and Goasguen JE
- Subjects
- Aged, Aged, 80 and over, Anemia, Refractory blood, Anemia, Refractory mortality, Anemia, Refractory pathology, Anemia, Sideroblastic blood, Anemia, Sideroblastic mortality, Anemia, Sideroblastic pathology, Humans, Middle Aged, Prognosis, Anemia, Refractory classification, Anemia, Sideroblastic classification
- Abstract
Clinical, haematological and outcome data were studied in 84 patients with acquired idiopathic sideroblastic anaemia (AISA) from a registry of 613 consecutive myelodysplastic syndromes (MDS) recorded by five institutions in western France. Two groups could be identified and compared: 'pure' erythroblastic AISA (AISA-E: 59 pts), and AISA with myelodysplastic features, i.e. dysgranulo and/or dysmegakaryopoiesis (AISA-M: 25 pts). Results were also compared to those of a series of 71 cases of refractory anaemia without sideroblastosis (RA) carried out from the same registry. Dyserythropoiesis was present in 90% of all AISA subtypes, dysgranulopoiesis in 88% of the AISA-M cases; dysmegakaryopoiesis was observed in 44% of AISA-M. Ten patients with both forms of AISA showed high platelet counts. These cases appeared particular in that four of them were associated with a splenomegaly and/or a hyperleucocytosis. They had to be distinguished from myeloproliferative syndromes. Outcome comparison of AISA-E with AISA-M showed a significant discrepancy of survival duration (60 vs 38 months respectively). Progression towards refractory anaemia with excess of blasts or acute leukaemia, was significantly higher for AISA-M than for AISA-E. The risk of transformation increased to 24% for the AISA-M group similarly to those of RA patients (17%). We conclude that AISA must be divided into two categories, 'pure' AISA and AISA-M, because survival duration and risk of transformation are different.
- Published
- 1992
- Full Text
- View/download PDF
21. Difference of bone marrow adipocyte colony-forming capacity between aplastic anemia and iron deficiency anemia.
- Author
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Hirata J, Umemura T, Kaneko S, Nishimura J, Motomura S, and Ibayashi H
- Subjects
- Anemia, Refractory, with Excess of Blasts pathology, Colony-Forming Units Assay, Humans, Adipose Tissue pathology, Anemia, Aplastic pathology, Anemia, Hypochromic pathology, Anemia, Refractory pathology, Bone Marrow pathology
- Abstract
The bone marrow adipocyte colony-forming capacity (Adipo-CFC) in patients with aplastic anemia (AA), myelodysplastic syndrome (MDS) and iron deficiency anemia (IDA) was studied. Before treatment, Adipo-CFC in IDA was higher than that in AA and MDS. After treatment, Adipo-CFC decreased in IDA, but it increased in AA and MDS only at the responsive stage. In this context, it is suggested that increase of Adipo-CFC occurs during not only regenerating hemopoiesis but also accelerated erythropoiesis such as severe IDA. Colony-stimulating activity (CSA) production by marrow stromal cells (MSC) in AA was lower than that in normal subjects. Low Adipo-CFC and defective CSA production by MSC may explain in part the pathogenesis of microenvironmental defect in AA.
- Published
- 1988
- Full Text
- View/download PDF
22. Correlation of bone marrow colony growth in the myelodysplastic syndromes with the FAB classification and the Bournemouth score.
- Author
-
Oscier DG, Worsley A, Darlow S, Figes A, Williams JD, and Hamblin TJ
- Subjects
- Anemia, Refractory pathology, Anemia, Refractory, with Excess of Blasts pathology, Anemia, Sideroblastic pathology, Colony-Forming Units Assay, Humans, Leukemia, Myelomonocytic, Chronic pathology, Myelodysplastic Syndromes classification, Bone Marrow pathology, Hematopoietic Stem Cells pathology, Myelodysplastic Syndromes pathology
- Abstract
Erythroid and myeloid colonies were grown from the bone marrow of 81 patients with myelodysplasia and the median number of colonies correlated with the FAB classification and Bournemouth score. CFU-GM were increased in CMML compared to RAEB and RAEBt. BFU-E were higher in RA than in the other FAB subgroups. Patients with a high Bournemouth score had poorer CFU-GM and BFU-E growth than those with a low score.
- Published
- 1989
- Full Text
- View/download PDF
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