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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recent data suggest that myeloid neoplasms are organized hierarchically in terms of self-renewal and maturation of early progenitor cells, similar to normal myelopoiesis. In acute myeloid leukemia (AML), the NOD/SCID mouse-repopulating leukemic stem cells usually co-express CD123 with CD34, but lack CD38. So far, however, little is known about expression of other markers and targets on these progenitors. In the present study, expression of target antigens on CD34+/CD38- cells was analysed by multi-color flow cytometry in patients with AML (n = 18), myelodysplastic syndromes (MDS, n = 6), chronic myeloid leukemia (CML, n = 8) and systemic mastocytosis (SM, n = 9). The IL-3Ralpha chain (CD123) was found to be expressed on CD34+/CD38- cells in a majority of the patients in all disease categories. Independent of the type of disease, the vast majority of these stem cells co-expressed aminopeptidase-N (CD13) and CD44 in all patients. By contrast, the CD34+/CD38- progenitor cells expressed variable amounts of the target receptor CD33, c-kit (CD117) and AC133 (CD133). In conclusion, neoplastic stem cells in various myeloid neoplasms appear to express a similar phenotype including target antigens such as CD13, CD33 and CD44. Since many of these targets are not expressed on all stem cells in all patients, the elimination of the entire clone may require combinations of targeted antibodies or use of additional drugs.
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PMID:Detection of molecular targets on the surface of CD34+/CD38-- stem cells in various myeloid malignancies. 1632 50

This study was aimed at exploring the immunophenotypic features of blasts in patients with myelodysplastic syndromes (MDS). Four-color flow cytometry using conventional and secondary gating strategies was used to immunophenotype blasts and the CD34 positive cells in bone marrow nucleated cells of 29 patients with MDS. The results showed: (1) with progression of MDS from RA/RAS, RAEB to RAEB-T, the proportion of CD34(+) cells were gradually increased from 8.0%, 46.4% to 76.8% (P < 0.05); (2) using CD45 vs SSC gating strategy, with the transformation of RA/RAS, RAEB to RAEB-T, the expression of HLA-DR, CD13, CD33, CD117 were also gradually increased (P < 0.05), and the expression of CD15 was gradually decreased (P < 0.05); (3) using CD45 vs CD34 gating strategy, the expression of HLA-DR, CD13, CD33, CD117 on blasts were higher by secondary gating method than those by conventional gating (P < 0.05). However, there were no significant difference (P > 0.05) in the expression of above-mentioned antigens on CD34(+) cells among different MDS subtypes. It is concluded that conventional gating method can reflect MDS progression from RA/RAS, RAEB to RAEB-T, and secondary gating strategy may accurately reflect the biological features of blasts in MDS. Abnormal expression of CD34 is related to the immaturity level and heterogeneity of blast cells, which is beneficial to the diagnosis of clinically suspected MDS incapable of diagnosing with morphology and cytogenetics.
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PMID:[Four-color flow cytometric immunophenotypic features of blasts in myelodysplastic syndromes]. 1658 91

We used a new method of data presentation and analysis, termed antigen mapping, to characterize recurring myeloblast phenotypic abnormalities in a series of 28 cases of myelodysplastic syndrome (MDS), including refractory anemia with ringed sideroblasts (RARS), refractory anemia with multilineage dysplasia (RCMLD), and refractory anemia with excess blasts (RAEB). Abnormal patterns of CD34 and CD117 expression were present in 50% of RARS, 68% of RCMLD, and 100% of RAEB cases. The presence of decreased myeloblast CD45 density, increased CD13 and CD34 density, and increased expression of CD11c and CD4(dim) were MDS grade-related. There was a direct relationship between the number of myeloblast phenotypic abnormalities (phenotypic score) and MDS grade. The myeloblast phenotypic scores also were correlated highly with International Prognostic Scoring System scores and risk categories. We found the antigen mapping technique to be an efficient data presentation and analysis method for the detection of MDS-associated abnormalities of antigen distribution and density.
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PMID:Myeloblast phenotypic changes in myelodysplasia. CD34 and CD117 expression abnormalities are common. 1669 Apr 88

Using a very simple flow cytometry protocol, we found that CD36 and CD117 on granulocytes and CD56 on monocytes were the major bone marrow phenotypic aberrations in patients with myelodysplasia, including CMML. CD56 on monocytes was associated with CMML. Importantly, phenotypic aberrations were lost on blood cells, except for CD56.
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PMID:A simple method for detection of major phenotypic abnormalities in myelodysplastic syndromes: expression of CD56 in CMML. 1755 Aug 65

The aim of this study was to investigate the clinical, pathological and biological features of biphenotypic acute leukemia. The morphology of tumor cells was observed by bone marrow examination; the immunophenotype was assayed by flow cytometry and immunohistochemistry; the chromosomal aberrations were detected by conventional chromosomal analysis and RT-multiplex nested PCR. The results showed that extramedullary skin lesions and myelodysplasia occurred before the onset of overt disease. At the time of diagnosis, this case had more than 30% blasts in bone marrow with meningeal involvement. Large-sized tumor cells predominated morphologically over other cells. Flow cytometry revealed the co-expression of myeloid antigens (cMPO, CD33 and CD117) and T-lymphoid antigens (cCD3, CD5, CD7, dual expression of CD4 and CD8). Immunohistochemical staining showed that CD43 and CD99 were strong positive which define the earliest hematopoietic progenitors. Partial tandem duplication of the MLL gene could be detected with normal cytogenetic method. All above-mentioned results led to the diagnosis of biphenotypic acute leukemia. It is concluded that the biphenotypic acute leukemia is an uncommon type of leukemia which may be preceded by myelodysplastic syndrome and has aggressive clinical and biological behavior. Immunophenotype, cytogenetics and molecular analysis can contribute to early diagnosis of BAL and evaluation of prognosis.
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PMID:[Skin lesions and myelodysplastic syndrome as initial manifestations of biphenotypic acute leukemia]. 1795 70

Acute myeloid leukaemia (AML) with multilineage dysplasia (MD) is one of the four main categories of AML in the World Health Organization (WHO) classification. The role of bone marrow trephine biopsy (BMTB) histology and immunohistochemistry in the diagnosis of AML-MD is currently unclear. BMTBs were studied in 11 cases of AML-MD and two cases of myelodysplasia that subsequently transformed to AML. Among them, six cases showed trilineage dysplasia and seven showed bilineage dysplasia. With respect to conforming to the WHO definition of AML, documentation of an increased proportion of immature myeloid cells was possible on morphology and counting of immature cells following immunostaining with CD34, CD117 or HLA-DR antibodies. Recognition and quantification of dysplastic features in the haemopoietic lineages was made easier by immunohistochemistry with antibodies to ret40f (glycophorin C), myeloperoxidase, CD61 and/or CD42b, CD34, CD117 and HLA-DR. Based on this relatively small series of cases we show the utility of BMTB and immunohistochemistry as an aid to the diagnosis of AML-MD. This has to be seen not just in light of its utility at diagnosis, but also the role the diagnostic BMTB would play for purposes of comparison when follow-up BMTBs are submitted in this group of patients.
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PMID:Bone marrow trephine findings in acute myeloid leukaemia with multilineage dysplasia. 1797 48

Occurrence of phenotypic abnormalities in CD34(+) hematopoietic progenitor and precursor cells (HPC) and their major B-cell and nonlymphoid compartments has been frequently reported in myelodysplastic syndromes (MDS). Here, we analyze for the first time the numerical and phenotypic abnormalities of different maturation-associated subsets of bone marrow (BM) CD34(+) HPC from 50 newly diagnosed MDS patients in comparison to normal/reactive BM (n=29). Our results confirm the existence of heterogeneously altered phenotypes among CD34(+) HPC from MDS and indicate that such variability depends both on the relative distribution of the different subsets of CD34(+) HPC committed into the different myeloid and B-lymphoid compartments, and their immunophenotype (for example, higher reactivity for CD117 and CD13 and lower expression of CyMPO, CD64 and CD65 on CD34(+) immature and neutrophil precursors), a clear association existing between the accumulation of CD34(+) HPC and that of immature CD34(+) HPC. Interestingly, expansion of erythroid- and neutrophil-lineage CD34(+) cells is detected in low-grade MDS at the expense of CD34(+) plasmacytoid dendritic cell and B-cell precursors, while expansion of immature CD34(+) precursors occurs in high-grade MDS. On the basis of the number and severity of the phenotypic abnormalities detected, a scoring system is proposed that efficiently discriminates between normal/reactive and MDS CD34(+) HPC, the mean score significantly increasing from low- to high-grade MDS.
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PMID:The immunophenotype of different immature, myeloid and B-cell lineage-committed CD34+ hematopoietic cells allows discrimination between normal/reactive and myelodysplastic syndrome precursors. 1833 65

This study was aimed to investigate the immunologic characteristics of refractory anemia with excess blasts-II (RAEB-II) which belongs to a new subtype of World Health Organization (WHO) classification of myelodysplastic syndrome (MDS) and to screen out the independent immunologic prognostic factors of MDS. 35 cases of adult patients with de novo MDS were investigated. The immunofluorescent analysis by multiparameter flow cytometry was performed at the double gating of CD45/SSC to determine the immunophenotype of MDS cells in all cases. All patients were followed up. 47 cases of acute myeloid leukemia (AML) M1, 51 cases of AML-M(2) and 38 cases of acute lymphocytic leukemia (ALL) were selected as control. Software SPSS 13.0 was applied to analyze all the related data. The results showed that the positive expression rate of HLA-DR in RAEB-II was 100%, which was high in sensitivity and specificity. CD13 (94.74%), CD33 (84.21%) and CD117 (78.95%) were also highly expressed in RAEB-II. CD13 in RAEB-II was significantly higher than that in refractory cytopenia with or without multilineage dysplasia (RA/RCMD) (p < 0.01) and REAB-I (p < 0.05); CD33, CD117 (p < 0.05) and stem cell antigen CD34 (p < 0.01) in RAEB-II were significantly higher than that in RCMD (p < 0.01), but no statistically significant difference was found as compared with RAEB-I (p > 0.05). Compared with AML-M(1) and AML-M(2), no significant difference of CD13 and CD117 in RAEB-II was found (p > 0.05). CD33 (p < 0.01) and CD34 (p < 0.05) were significantly lower than that in AML-M(1), but no significant difference was found as compared with AML-M(2) (p > 0.05); CD15 (p < 0.01) and CD11b (p < 0.05) was significantly lower than that in M(2), but no significant difference was found as compared with AML-M(1) (p > 0.05); MPO was significantly lower than that in AML-M(1) and M(2) (p < 0.05); HLA-DR was significantly higher than that on AML-M(2) (p < 0.05), but no significant difference was found as compared with AML-M(1) (p > 0.05). RAEB-II did not express CD2, CD3, CD5 and CD8 (positive rate 0%, p < 0.01) when compared with T-ALL; CD4 (p < 0.05) and CD7 (p < 0.01) were significantly lower than that in T-ALL. RAEB-II did not express CD19 and CD20 (positive rate 0%, p < 0.01) as compared with B-ALL; CD10, CD22 and cCD79a were significantly lower than that in B-ALL (p < 0.05). CD117 (p = 0.0197) and MPO (p = 0.0085) were the two prognostic immunological antigens as regards the overall survival (OS) of MDS; CD117 (p = 0.003) was the single parameter in Cox regression. It is concluded that RAEB-II expresses mainly myeloid antigen without or with little expression of lymphoid antigen. Unique individual immunophenotypic features can be detected in patients with RAEB-II. HLA-DR can be a specific parameter to distinguish the other subtypes of MDS. CD117 may be an independent prognostic immunological antigen as regards OS of MDS.
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PMID:Immunologic characteristics and prognosis of myelodysplastic syndrome new subtype: refractory anemia with excess blasts-II. 1923 59

A new myeloid leukemia cell line (CG-SH) with normal cytogenetics was established from a patient with acute myelogenous leukemia (AML) following myelodysplastic syndrome (MDS). The cells of CG-SH are immature blasts and have an immature myeloid phenotype (positive for myeloperoxidase, CD7, CD34, CD38, CD117, HLA-DR, negative for CD10, CD19, CD20, CD41, CD42). A partial expression of CD13, CD15, CD65 and a weak expression of CD33 and CD133 was noted. The cells are negative for EBER. By molecular analysis, a mutation of NRAS and heterozygous mutations of RUNX1 were detected. No mutations were detected in FLT3-ITD, MLL-PTD or NPM1. By real-time PCR, a series of 19 microRNAs was identified which are strongly expressed in CG-SH. In conclusion, a new cell line was established which will be useful for the study of AML with normal cytogenetics and mutations in NRAS and/or RUNX1.
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PMID:Characterization of a new myeloid leukemia cell line with normal cytogenetics (CG-SH). 1941 91

Bone marrow (BM) hematopoietic progenitor cells (CD34+) are a heterogeneous population with varying degrees of commitment and maturation to several cell lineages. In myelodysplastic syndromes (MDS), this population is increased. We examined the major cell types found in the blast gate by flow cytometry in newly diagnosed patients with MDS, compared them to normal BM and studied their variation according to WHO type. Two subsets defined by SSC were found both in normal BM and MDS, corresponding to myeloblasts and B-cell precursors. The number of B-cell precursors among all nucleated cells was equally low, independent of WHO type. However, the subset with an intermediate SSC, but CD117, CD13 and CD19 negative increased with the rise of myeloblasts. Concomitantly, the ratio between CD34+/CD117+/CD34-/CD117+ cells was increased. These two features are consistent with the maturation block occurring in the progression of the neoplastic clone. We conclude that the quantitative analysis of the cell types present in the BM blast gate by flow cytometry is not only important for the diagnosis of MDS in patients with peripheral cytopenias and a normal karyotype, but gives also important prognostic information of the patients.
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PMID:Variation of bone marrow CD34+ cell subsets in myelodysplastic syndromes according to who types. 1958 Mar 46


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