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

Two cases of unclassified chronic myeloproliferative disorders (UCMPD), diagnosed by hematological, cytogenetic and DNA analyses, are described. Case 1: a 63 year old female was admitted because of leukocytosis (96,800/microliters) and splenomegaly. Hematological examinations revealed an increase of the granulocytes in the peripheral blood and bone marrow. The neutrophil alkaline phosphatase (NAP) score was 121. The patient developed blast crisis after 12 months of the chronic phase. Case 2: a 48 year old male was presented with fever and leukocytosis (20,000/microliters). Hematological examinations revealed an increase of granulocytes in the peripheral blood and bone marrow. The NAP score was 33. Maturation-arrest in granulocytic series and morphological abnormalities of marrow cells were not observed in the two cases. Cytogenetic analysis of bone marrow cells disclosed 46, XX, i (17 q) in case 1 and 47, XY, +8 in case 2. Southern blot analysis using 3' bcr probe and TransProbe-1 showed no bcr rearrangement. These cases are thought to be valuable in order to clarify the relationship between UCMPD and CMPD such as Ph1 negative chronic myelocytic leukemia and myelodysplastic syndromes.
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PMID:[Two cases of unclassified chronic myeloproliferative disorders]. 160 19

The activity of alkaline phosphatase and peroxidase was measured in polymorphonuclears in 20 cases of myelodysplasia syndromes, 10 cases of chronic myeloid leukaemia. Reduced phosphatase activity was found in 5 cases and peroxidase activity in 3 cases of myelodysplasia syndromes. No evident correlation was noted between the activity of these enzymes and prognosis. Increased proportion of peroxidase-negative granulocytes was observed, moreover, in most cases of chronic myeloid leukaemia. The observations will be continued in larger material.
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PMID:[Results of selected cytochemical studies of mature granulocytes in myelodysplastic syndromes and various other hematologic diseases]. 182 68

Myelodysplastic syndromes (SMD) were studied in 58 patients (37 men, 21 women; mean age 61 years, range 18-81) who were grouped according to FAB criteria (Table 1). None of them showed a secondary SMD to medullary toxic agents or cytostatic treatments although 5 presented concomitant neoplastic disease. Morphologic alterations in peripheral blood smears and bone marrow were registered by 3 hematologists working independently. The intracellular and extracellular iron deposits were evaluated in every case with Perls; peroxidase activity was determined in 16 patients and intraleucocitary alkaline phosphatase reaction was carried out in 17 patients. Twenty five patients (43%) had refractory anemia (RA); 10 (17%) sideroblastic anemia; 13 (25%) refractory anemia with excess of blasts (AREB); 3 (5%) AREB in transformation (AREB-T) and 7 myelomonocytic leukemia (LMMC). Clinical manifestations at diagnosis are described in Table 2. In the observation period there were cases of anemia requiring transfusion, bacterial infections, muco-cutaneous hemorrhage and hemorrhagic episodes in the central nervous system. In the bone marrow smears the cellularity was normal or increased in 53 cases and diminished in only 3. The degree of dysplastic characteristics (erythroid, granulocytic and megakaryocytic) ranged from low to severe. It was low in most of AR, being the erythroid population the most affected in AS and the granulocytic one in AREB and AREB-T. Patients with LMMC showed similar characteristics to those with myeloproliferative syndromes and the differential diagnosis were sometimes difficult, accounting for their separate inclusion in Table 4. Out of 23 patients, 5 presented clonal pathology detected in cytogenetic studies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Myelodysplastic syndrome: experience of the Study and Treatment of Bone Marrow Failure Group]. 213 Feb 4

This study examines the effect of cytosine arabinoside (Ara-C) on CFU-GM progenitor cells grown in methylcellulose culture from normal and myelodysplastic subjects and patients with acute non-lymphoblastic leukaemia. Light density marrow cells were incubated during culture with Ara-C concentrations ranging from 10(-4) M to 10(-12) M. After counting, colonies were cytospun and cells within the colonies examined for alkaline phosphatase positivity and expression of HLA-DR antigen, as indices of differentiation. Monocytes/macrophages were also enumerated in colonies using the monoclonal antibody CD14. In all subjects, 10(-4) M to 10(-6) M Ara-C caused significant reduction in CFU-GM colony formation compared with control (no Ara-C). In no instance did colony numbers increase. Ara-C across the dose curve had no effect on myeloid differentiation markers in any of the groups studied. Similarly, percentages of CD14 positive cells in colonies were not altered by exposure to Ara-C. Using this clonogenic model, these data suggest that Ara-C does not induce differentiation of CFU-GM stem cells in normal subjects or patients with myelodysplasia/acute non lymphoblastic leukemia.
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PMID:Cytosine arabinoside does not cause differentiation in vitro of CFU-GM in marrow from normal, myelodysplastic or ANLL subjects. 231 14

23 patients with myelodysplastic syndromes (MDS) and 8 normal controls were analyzed for dysmegakaryopoiesis (DMP) in the bone marrow by alkaline phosphatase anti-alkaline phosphatase (APAAP) technique and by conventional May-Giemsa staining. In the immunocytochemical study, monoclonal antibody (MoAb) against glycoprotein (GP) IIb/IIIa was utilized to demonstrate megakaryocytic cells. 91% (21/23) of MDS cases were detected as having DMP by APAAP method, while only 52% (12/23) were detectable by Giemsa stain. There were difficulties in recognizing small micromegakaryocytes (micro MKs), designated as type 1 atypical MKs, by Giemsa staining. Furthermore, megakaryoblasts (MKBs) were detectable only by APAAP technique. In 8 normal controls, no type 1 and type 3 atypical MKs (round shaped multinuclear MKs) were observed either by Giemsa staining or by the APAAP method, suggesting that they are a distinctive feature of MDS. These results indicate the necessity of immunocytochemical technique for accurate recognition of DMP in MDS.
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PMID:Comparative study of immunocytochemical staining versus Giemsa stain for detecting dysmegakaryopoiesis in myelodysplastic syndromes (MDS) 231 99

We examined the intracellular alkaline phosphatase (NIAP) activities in peripheral neutrophils in 15 normal controls, 4 patients with myelodysplastic syndrome, and 4 with chronic myeloid leukemia. NIAP activities were decreased in myelodysplastic syndrome in comparing to normal controls (p less than 0.01). These data suggest that measurement of NIAP activity is useful for supporting a diagnosis of myelodysplastic syndrome.
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PMID:[Intracellular alkaline phosphatase activities in myelodysplastic syndrome]. 234 69

Neutrophils and band forms from patients with acute myeloid leukemia and myelodysplastic syndrome were stained for the presence of myeloperoxidase using a cytochemical method (diaminobenzidine/hydrogen peroxide) and the alkaline phosphatase--anti-alkaline phosphatase immunocytochemical procedure (using monoclonal anti-myeloperoxidase). Neutrophils and bands were also stained for elastase and lactoferrin using monoclonal and polyclonal antibodies, respectively. Subpopulations of neutrophils and bands from cases of acute myeloid leukemia and myelodysplasia exhibited a qualitative and/or quantitative deficiency in myeloperoxidase. In addition, a quantitative decrease in elastase and/or lactoferrin staining was detected. Thus, neutrophils and bands from patients with acute myeloid leukemia and myelodysplastic syndrome have a defect in one or more of the constituents of primary and/or secondary granules. These defects are consistent with the view that abnormal neutrophils and bands are derived from a malignant clone of myeloid precursor cells.
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PMID:Abnormal neutrophils in acute myeloid leukemia and myelodysplastic syndrome. 283 2

In 45 cases of primary myelodysplastic syndrome; 16 refractory anaemia (RA), 11 RA with ring sideroblasts (RA+), 13 RA with excess of blasts (RAEB), 5 chronic myelomonocytic leukaemia (CMML), the relations between myeloperoxidase (MPO) activity in polymorphonuclear leucocytes (PMN), neutrophil alkaline phosphatase (NAP) activity, absolute number of PMN and thrombocytopenia were investigated. 11 patients (26%) showed abnormal numbers (greater than 4%) of MPO-deficient PMN and 27 (75%) showed abnormal NAP activity (NAP score; greater than 134.0, less than 15.0), mostly decreased. No significant correlations between MPO activity and NAP activity were demonstrated, nor were any significant correlations found with the other parameters investigated. The FAB-subtypes, RAEB and CMML, showed a significant correlation to thrombocytopenia (p = 0.028) and to pancytopenia (p = 0.024). The findings may support the view that at least some of the myelodysplastic syndromes may be fundamentally the same disease as acute myeloid leukaemia.
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PMID:Myeloperoxidase-deficient polymorphonuclear leucocytes. (V): Relation to FAB-classification and neutrophil alkaline phosphatase activity in primary myelodysplastic syndromes. 299 23

We examined the in vitro effect of recombinant human granulocyte colony-stimulating factor (rhG-CSF) on neutrophil anomalies in 20 patients with myelodysplastic syndromes (MDS) and eight patients with chronic myelogenous leukemia (CML). Neutrophil alkaline phosphatase (NAP) activity was determined in nine MDS patients and eight CML patients by a scoring method. NAP scores were decreased in six of the nine patients with MDS and in all of the patients with CML. In all patients with these diseases, NAP scores increased by incubating the blood with rhG-CSF. An increase in NAP scores by rhG-CSF was observed even at a concentration of 1 U/mL in patients with MDS but was observed only at higher concentrations (1,000 to 10,000 U/mL) in patients with CML. Significant increases in NAP scores occurred at 12 hours' incubation in patients with MDS, whereas the increase was more gradual in patients with CML. This time course difference was thought to be due mainly to the difference in cell populations of circulating myeloid cells between MDS patients and CML patients. Induction of NAP activity by rhG-CSF in patients with both these diseases was suppressed by the addition of inhibitors of RNA or protein synthesis. Neutrophil superoxide anion (O2-) production induced by N-formyl-methionyl-leucyl-phenylalanine (fMLP) was determined in the other 11 patients with MDS. This neutrophil function was decreased in seven of the 11 patients with MDS, normal in two patients, and increased in two patients. Preincubation with rhG-CSF caused a significant increase in fMLP-induced O2- production in nine of the 11 patients with MDS. rhG-CSF enhanced this neutrophil function in a time- and dose-dependent manner, and maximal stimulation was observed at 2,000 to 4,000 U/mL of rhG-CSF and at five to ten minutes' incubation. The present results show that rhG-CSF is able to repair at least in part the neutrophil anomalies in these patients, and our data, especially for patients with MDS, suggest the clinical usefulness of rhG-CSF for this preleukemic disorder.
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PMID:Recombinant human granulocyte colony-stimulating factor repairs the abnormalities of neutrophils in patients with myelodysplastic syndromes and chronic myelogenous leukemia. 303 12

The alkaline phosphatase-antialkaline phosphatase (APAAP) immunocytochemical staining technique was used to look for circulating cells of megakaryocyte lineage in peripheral blood smears from 67 cases of myelodysplasia. Small numbers of micromegakaryocytes positive for platelet glycoprotein IIIa were found in 23 cases. These cells superficially resemble small lymphoid cells and are hence difficult or impossible to recognise in conventional Romanowsky stained smears. Circulating micromegakaryocytes were found most commonly in more aggressive types of myelodysplasia (such as refractory anaemia with excess blasts (RAEB) and refractory anaemia with excess blasts in transformation (RAEB-t], and their presence may therefore indicate a poor prognosis. Because of the simplicity of this immunocytochemical labelling technique, it could be of wide use in the initial assessment of patients with myelodysplasia, and possibly for the early detection of acute leukaemic transformation.
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PMID:Circulating micromegakaryocytes in myelodysplasia. 332 97


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