Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The separate effects of vitamin D3 (1,25(OH)2D3) and 13-cis retinoic acid on the differentiation in liquid culture of marrow cells from seven patients with myelodysplastic syndrome (MDS) were studied. Following incubation with 1,25(OH)2D3, an increasing number of myeloid cells acquired the morphological appearance of mature monocyte-macrophages and reacted positively to fluoride-sensitive naphthyl acetate esterase and specifically bound My4 monoclonal antibody (McAb). Incubation of bone marrow cells with 13-cis retinoic acid enhanced the number of cells with the morphological appearance of metamyelocytes and mature granulocytes as well as those that reacted positively with AS-D naphthol chloroacetate esterase. The results suggest that the differentiation pattern of myeloid precursor cells from MDS patients can be modulated by 1,25(OH)2D3 and 13-cis retinoic acid.
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PMID:Differentiation of bone marrow cells from myelodysplastic patients in the presence of 1,25 dihydroxyvitamin D3 or 13-cis retinoic acid. 309 42

gamma-Interferon (IFN-gamma) has previously been found to induce monocytic differentiation in established leukemic cell lines, such as HL-60 and U937. The aim of the present study was to evaluate the differentiative effect of highly purified recombinant (r)IFN-gamma on fresh bone marrow cells from patients with acute nonlymphocytic leukemia (n = 11) or myelodysplastic syndromes (n = 3). Blast cells were cultured in suspension in the presence or absence of rIFN-gamma (10-10(3) U/ml). While 6 out of 14 cases were unresponsive to rIFN-gamma in vitro, the remaining 8 patients showed a significant increase (0.05 greater than p greater than 0.001) in the percentage of cells expressing C3bi receptors, detected by OKM1 (median value in control cell, 9.5; median value in rIFN-gamma-treated cells, 31) and Mo1 (8.5 vs. 36), and in the percentage of cells expressing the monocytic antigens detected by Mo2 (8 vs. 28) and MY4 (6.5 vs. 32.5). In the responsive patients morphologic changes consistent with monocytic maturation, as well as a strong increase of alpha-naphthyl acetate esterase activity and of nitroblue tetrazolium reducing capability were observed upon culture with rIFN-gamma. We conclude that (a) rIFN-gamma may induce in vitro monocytic differentiation of blasts from acute nonlymphocytic leukemia and myelodysplastic syndrome patients, and that (b) this agent should be investigated for its capacity to be active in vivo.
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PMID:Recombinant gamma-interferon induces in vitro monocytic differentiation of blast cells from patients with acute nonlymphocytic leukemia and myelodysplastic syndromes. 312 8

Cancer and Leukemia Group B demonstrated that adults with acute lymphoid leukemia (ALL) possessing blast cells with myeloid antigens (My+ALL), as identified by monoclonal antibodies against CD13 and CD33, have a worse prognosis than those lacking myeloid antigens (My-ALL). Consequently, we further studied this group of adults with ALL to determine if these immunological groups could be distinguished by morphological and cytochemical criteria. Bone marrow films were classified according to French-American-British Co-operative Group Criteria, assessed for myelodysplasia, and examined for blasts with azurophilic granules. More cases of My+ALL had L2 morphology than did My-ALL (68% vs. 49%, p = 0.04), and more cases of My+ALL were positive for acid alpha-naphthyl acetate esterase (61% vs. 31%, p = 0.03). The presence of myelodysplastic changes was not significantly different in My+ALL (13%) as compared to My-ALL (5%), but more cases of My+ALL had unusual blasts (monocytoid features and cytoplasmic buds) than did My-ALL (19% vs. 0%, p less than 0.01). In addition, more cases of My+ALL had greater than 5% of the blasts with azurophilic granules (42% vs. 13%, p = 0.01). In the My+ALL group the presence of azurophilic granules was associated with a longer median survival (13.5 months vs. 1.5 months, p less than 0.01). We conclude that My+ALL can be suspected when cases possess L2 morphology, unusual blasts, positive staining for acid alpha-naphthyl acetate esterase, and greater than 5% azurophilic granules. In addition, the poor risk group (My+ALL) can be further subdivided into better and poorer risk subgroups based on the presence of azurophilic granules.
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PMID:Morphologic and cytochemical characterization of adult lymphoid leukemias which express myeloid antigen. 316 98

By using the combination of alpha-naphthyl butyrate esterase and chloroacetate esterase for cytochemical detection of monocytes and granulocytes, respectively, we examined and identified five adult patients with acute myeloid leukemia whose leukemic cells often (20 to 30%) had the characteristics of both monocytes and granulocytes. All five patients were men, 23 to 82 years of age. Two patients had manifestations of preleukemia. One of these two patients had received treatment for lymphoma for 12 months before acute myelomonocytic leukemia was diagnosed. One patient had gum hypertrophy, and two had leukemia cutis. At the time of initial examination, four patients had blood leukocyte counts higher than 80,000/mm3 and one had leukopenia. Four patients received chemotherapy; three responded temporarily but died within 1 year after the myelomonocytic leukemia had been diagnosed. The patient with leukopenia has remained in complete remission for 2 years. With more frequent use of double esterase stains for classification of acute myeloid leukemias, this variant of acute myelomonocytic leukemia should be detected more often and its clinical behavior should be better understood.
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PMID:Acute myelomonocytic leukemia: an unusual variant with both granulocytic and monocytic esterases in the leukemic cells. 345 74

Although it is well recognized that granulocytic sarcoma can cause localized lymphadenopathy, widespread nodal involvement by acute myelocytic leukemia (AML), clinically mimicking non-Hodgkin's lymphoma, has only been previously described twice. We report the clinicopathological, immunological, and cytochemical features of two patients who had widespread, prominent lymphadenopathy secondary to AML as well as concurrent marrow leukemia (M1 and M2). For one patient the lymphadenopathy was the predominant abnormality prompting him to seek medical attention, while the second patient had symptoms of infection following a 9-month history of myelodysplasia. The disease in both patients was aggressive; one patient survived only 1 week and the other survived only 5 weeks after diagnosis. In both cases the granulocytic sarcoma was confirmed by cytochemistry studies (naphthol ASD-chloroacetate esterase on tissue sections and myeloperoxidase on imprint smears), and electron microscopy, including morphology (both cases) or ultrastructural localization of myeloperoxidase (case 2). Non-specific esterase activity was not detected in either patient's blasts, although serum lysozyme was elevated in both cases. Immunological studies revealed reactivity of both patients' cells with panleukocyte, MY4, MY7, OKM-1, and Leu-M1 monoclonal antibodies and with alpha-1-antitrypsin and muramidase antibodies. The cells of one of these patients also reacted with anti-S-100 protein. Although the cytochemical studies indicated that both cases exhibited only myeloid differentiation, the immunological markers suggested that the tumor cells possessed some features of monocytes, perhaps explaining their propensity for widespread tumor formation. Morphological, immunological, cytochemical, and ultrastructural methods of diagnosing granulocytic sarcoma are presented.
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PMID:Acute myelocytic leukemia manifested by prominent generalized lymphadenopathy: report of two cases with immunological, ultrastructural, and cytochemical studies. 345 62

Chronic myelomonocytic leukemia (CMML) is a rare leukemia, which is now included in myelodysplastic syndromes. In a small number of patients with CMML, problems in the diagnosis have been reported, especially when atypical morphological features in both monocytic and granulocytic cells due to dysmyelopoiesis are prominent, or when cytochemical characteristics are lost in the leukemic cells. The case history of a sixty-seven year-old male patient with CMML is described. The diagnosis of CMML in the patient was supported by the following evidence: chronic course of his disease; increased monocyte-like cells without other cause; normocytic anemia; immature granulocytic cells with hypogranular feature and giant platelets were observed in the peripheral blood. The bone marrow showed myeloid hyperplasia. Serum muramidase and vitamin B12 levels were increased, while neutrophil alkaline phosphatase score was low in the peripheral blood. Ph' chromosome was negative. The monocyte-like cells completely lacked nonspecific esterase. However the cells were confirmed as monocytic cells by flow cytometry using monoclonal antibodies to monocytes (OKM5).
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PMID:Report of a case with chronic myelomonocytic leukemia: demonstration of leukemic monocytes lacking nonspecific esterase by flow cytometry using monoclonal antibodies. 350 52

The effect of low dose (10(-12)-10(-7) M) ARA-C on differentiation and proliferation in liquid and semisolid culture of marrow cells from 13 patients with myelodysplastic syndrome (MDS) were studied following incubation in liquid culture with low dose ARA-C. In six of ten patients an increasing number of myeloid cells acquired the morphologic appearance of mature monocyte-macrophages. Increasing number of cells reacted positively to fluoride sensitive naphthyl acetate esterase and specifically bound MY4 monoclonal antibody. Phagocytosis and killing of Candida albicans by monocyte-macrophages incubated with low dose ARA-C was normal and similar to that of the untreated cells. All MDS patients showed reduced myeloid colony and increased cluster formation. Low dose ARA-C had slight but non-significant inhibitory effects on myeloid colony growth. The results indicated that the differentiation pattern of myeloid precursor cells form a subset of MDS patients was altered by exposure to low dose ARA-C in vitro.
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PMID:The effect of low dose ARA-C on in-vitro haemopoiesis of marrow cells from myelodysplastic patients. 356 Oct 19

Quantitative cytochemistry of components of blood neutrophil azurophilic granules (myeloperoxidase, chloroacetate esterase, beta-glucuronidase, and acid phosphatase) and specific granules (lactoferrin) has been performed by scanning and integrating microdensitometry in 13 patients with a myelodysplastic syndrome and 11 patients with chronic granulocytic leukaemia. Both patient groups showed a reduction of enzyme activity in azurophilic granules, and also of lactoferrin, consistent with abnormal development of neutrophil granules. These cytochemical changes in blood neutrophils are similar to those found in acute myeloid leukaemia, are consistent with a leukaemic maturation defect, and may be of diagnostic value.
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PMID:Quantitative cytochemistry of blood neutrophils in myelodysplastic syndromes and chronic granulocytic leukaemia. 609 32

Acid alpha naphthyl acetate esterase (ANAE) and combined ANAE-chloroacetate esterase cytochemistry was performed on 121 bone marrow aspirates from primary myelodysplastic syndromes (MDS) and a secondary dysplasia-megaloblastic anaemia (MA). The investigation demonstrated the presence of abnormal ANAE positive granulocyte populations in a significant proportion of cases. These cells, in which the staining patterns were characterized by atypical granular ANAE positivity and double ANAE-chloroacetate reactions, were shown immunologically to lack the receptor and antigenic characteristics of monocytes and morphologically to be granulocytes. Isoelectric focusing, however, indicated that the atypical esterase cytochemistry of these granulocytes was due to the presence of markedly increased concentrations of ANAE isoenzymes usually found in monocytes. Atypical ANAE-staining granulocytes were particularly evident in MDS marrows showing sideroblastic erythroid changes, whilst in MA they were mainly seen in cases of intermediate severity. It is suggested that these cells are associated with dysmyelopoietic changes in both malignant and non-malignant conditions.
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PMID:Esterase cytochemistry in primary myelodysplastic syndromes and megaloblastic anaemias: demonstration of abnormal staining patterns associated with dysmyelopoiesis. 658 35

A novel human cultured cell line, P39/Tsugane, was established from leukemic cells in the peripheral blood of a 69-year-old male with overt leukemia following myelodysplastic syndrome (MDS). P39/Tsugane cells were characterized by blastic appearance, presence of NaF-sensitive alpha-naphthyl butylate esterase activity, Fc gamma-receptor, C3-receptor, capacity to phagocytize sensitized erythrocytes, and reactivity with monoclonal antibodies such as OKT4, My4, VIMD5, MCS-2 and My7. These data indicate that P39/Tsugane cells are of myelomonocytoid nature. P39/Tsugane had a hypodiploid chromosome constitution with a gain of a consistent marker, 6q+, the presence of less consistent markers 9q+ and rcp(14;16), and random and non-random losses of autosomes: in accordance with the reported cytogenetic profiles of MDS, a representative karyotype of the present cell line is 45,XY,+del(6)(q15),9q+, t(14;16)-(q24;q21),-16,-17. P39/Tsugane cells were transplantable intraperitoneally into nude mice, and produced abdominal tumors and hemorrhagic ascites. These results indicate that P39/Tsugane is the first cultured cell line of myelomonocytoid nature to be derived from overt leukemia following MDS. Therefore, P39/Tsugane cells should be useful for studies on the differentiation of leukemia cells, the pathogenesis of MDS and in vitro-in vivo experimental chemotherapy.
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PMID:A novel human myelomonocytoid cell line, P39/Tsugane, derived from overt leukemia following myelodysplastic syndrome. 659 19


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