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)

Expression of P-glycoprotein (PGP), the product of the multi-drug resistance mdr1 gene was studied by immunocytochemistry on bone marrow slides using JSB1 monoclonal antibody and the alkaline phosphatase-antialkaline phosphatase (APAAP) and avidin-biotin-peroxidase (ABC) techniques in 82 cases of untreated myelodysplastic syndromes (MDS), of whom ten had evolved to AML (MDS-AML). The relationship between PGP expression, myeloperoxidase activity and immunophenotype of blast cells, karyotype and outcome was also analyzed. PGP expression was found in the blasts of 34 of the 82 patients (41%), the majority of blasts being stained in positive cases. PGP positivity was rare in 'low risk' MDS (RA and RARS: 2/12 cases) as opposed to 'high risk' MDS (RAEB, RAEB-T, CMML: 25/60 cases) and MDS-AML (7/10 cases) (p = 0.04). PGP expression was positively correlated to the presence of myeloperoxidase activity in less than 3% of blasts (p = 0.025), and CD34 antigen expression (p = 0.04), whereas CD33 antigen expression had borderline significance (p = 0.07), demonstrating that PGP expression predominated in blasts with an immature phenotype. An abnormal karyotype, and especially the presence of monosomy 7, was not correlated to a higher incidence of PGP expression, however. There was a trend for more frequent progression to AML and for shorter survival in PGP-positive cases, but differences with PGP-negative cases were not significant. Twenty patients received intensive anthracycline-Ara-C chemotherapy and ten (50%) achieved complete response, including 9/13 (69%) PGP-negative cases and 1/7 (14%) PGP-positive cases (p = 0.03). Twenty other patients were treated with low-dose Ara-C and ten (50%) responded (complete or partial response). PGP-positivity did not negatively affect response to low-dose Ara-C: 4/11 responses in PGP-negative, and 6/9 responses in PGP-positive patients (p = 0.18). Because the treatment choice in advanced MDS (especially between anthracycline-Ara-C or low-dose Ara-C, chemotherapy) is difficult, our preliminary therapeutic results suggest that the analysis of PGP expression could have practical importance in MDS. These findings however, will have to be confirmed on larger numbers of patients. Clinical trials using drugs potentially reverting mdr, activity could also be warranted in MDS.
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PMID:Expression of the multidrug resistance P-glycoprotein and its relationship to hematological characteristics and response to treatment in myelodysplastic syndromes. 751 32

Cytochemical and immunocytochemical studies were performed on bone marrow aspirates from 96 cases of primary myelodysplastic syndrome (MDS), 11 cases of secondary MDS, 22 cases of non-MDS hematologic disorders, and 10 cases of nondiagnostic, apparently normal marrow specimens to determine the practicality and utility of these stains for diagnosing MDS. Cytochemical studies included iron stain, periodic acid-Schiff (PAS), peroxidase, butyrate esterase, chloroacetate esterase, and double esterase stains. Immunocytochemical staining was done with monoclonal antibody HP1-1D, which recognizes the glycoprotein IIb/IIIa complex in megakaryocytes. The iron stain remained most helpful in identifying abnormal ringed sideroblasts, a feature of dyserythropoiesis, and thus in supporting the diagnosis of MDS. The PAS stain was helpful, if positive, in identifying patients with MDS; however, when it was negative, this stain did not help distinguish MDS from non-MDS hematologic disorders. The immunocytochemical stain with HP1-1D monoclonal antibody was also helpful in identifying atypical micromegakaryocytes, indicative of dysmegakaryopoiesis. Other cytochemical abnormalities were infrequently observed and were less specific for the diagnosis of MDS. The combination of two stains--for example, PAS and iron stain or PAS and double esterase--was helpful, however, in excluding MDS, inasmuch as neither the miscellaneous nor the control group stained positively with these combinations.
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PMID:Myelodysplastic syndrome: diagnostic implications of cytochemical and immunocytochemical studies. 767 45

We describe a patient with basophilic leukaemia following a 2-year period with myelodysplastic syndrome (refractory anaemia). The marrow showed 59.4% of blasts with 25.0% of mature and immature basophils. The leukaemic blasts contained granules, positively stained with toluidine blue but negative for peroxidase. The basophilic differentiation was confirmed by ultrastructural analysis demonstrating immature basophil granules. In addition, a morphological transition from immature blasts to more mature basophils was observed. Immunophenotypic analysis of blasts and basophils showed positive for CD5, CD7, CD13, CD33 and CD34. Cytogenetic investigation showed an abnormal karyotype, 46,XY,del(5)(q31q35), in 11% of the cells examined when the initial diagnosis of refractory anaemia was made. However, expansion of the same clone up to 100% was observed concomitantly with transformation to basophilic leukaemia.
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PMID:Transformation into acute basophilic leukaemia in a patient with myelodysplastic syndrome. 773 71

Cytogenetic data were studied in 26 patients with de novo acute myeloid leukemia (AML) with minimal myeloid differentiation, corresponding to the M0 subtype of the French-American-British classification, in correlation with cytoimmunologic and clinical findings. Clonal abnormalities were detected in 21 cases (80.7%), 12 of which had a complex karyotype. Partial or total monosomy 5q and/or 7q was found, either as the sole aberration or in all abnormal metaphases, in 11 patients; in 8 cases, additional chromosome changes were present, including rearrangements involving 12p12-13 and 2p12-15 seen in 3 cases each. Five patients had trisomy 13 as a possible primary chromosome change; in 5 cases, nonrecurrent chromsome abnormalities were observed. Comparison of these findings with chromosome data from 42 patients with AML-M1 shows that abnormal karyotypes, complex karyotypes, unbalanced chromosome changes (-5/5q- and/or -7/7q- and +13) were observed much more frequently in AML-M0 than in AML-M1. Patients with abnormalities of chromosome 5 and/or 7 frequently showed trilineage myelodysplasia and low white blood cell count. Despite their relatively young age, complete remission was achieved in 4 of 11 patients only. Patients with +13 were elderly males with frequent professional exposure to myelotoxic agents. Unlike patients with clonal abnormalities, most AML-M0 patients with normal karyotype showed 1% to 2% peroxidase-positive blast cells at light microscopy and frequently achieved CR. It is concluded that (1) AML-M0 shows a distinct cytogenetic profile, partially recalling that of therapy-related AML, (2) different cytogenetic groups of AML-M0 can be identified showing characteristic clinicobiologic features, and (3) chromosome rearrangements may partially account for the unfavorable outcome frequently observed in these patients.
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PMID:Cytogenetic profile of minimally differentiated (FAB M0) acute myeloid leukemia: correlation with clinicobiologic findings. 854 74

A 55-year-old female presented with sore throat and slight fever. The patient was admitted to our hospital on December 13, 1993. Full blood count showed hemoglobin 10.7 g/dl, white cell count 960/microliters (neutrophils 14%, lymphocytes 82%, blasts 2%) and platelets 13,000/microliters. Bone marrow examination showed hypocellularity with 4.5% of myeloblast positive for peroxidase. The bone marrow specimens on Dec. 20 showed 15.5% of myeloblasts, some of which had Auer rods. These findings led to the diagnosis of refractory anemia with excess myeloblast in transformation (RAEB-T) of French-American-British Cooperative Group. The patient was transfused and treated with cytarabine ocfosfate (SP-AC) (100 mg tid) and 6-mercaptopurine (50 mg tid) for 14 days. During chemotherapy she complained of nausea and anorexia, but they were managed easily with medication. On Feb. 7, 1994, forty-two days after the start of administration, peripheral blood and bone marrow aspirate were compatible with a complete remission. Although complete remission was sustained with courses of chemotherapy for 4 months, relapse occurred and the patient died of septicemia on August 29, 1994 after induction failure. Observation suggested that oral SPAC in combination with 6-mercaptopurine had a good antileukemic effect on the myelodysplastic syndrome. However, the duration response was short, and further improvement of the therapy is needed.
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PMID:[Refractory anemia with excess myeloblast in transformation induced remission by combined oral administration of cytarabine ocfosfate and 6-mercaptopurine]. 779 1

Nine patients with acute leukemia showing 10% or more positive blast cells with platelet peroxidase (PPO) or CD41b were diagnosed as megakaryoblastic leukemia. Three patients transformed from myelodysplastic syndromes or myeloproliferative disorders. The PPO positivity ranged from 7 to 55% (median 45%), and that for CD41b was 1.6 to 67.0% (median 16.4%). Because electron microscopic myeloperoxidase or glycophorin A were also positive in some patients, and also because CD41b positivity was often discordantly lower than PPO positivity, a possibility of mixed leukemia demonstrating myeloid or erythroid differentiation was suggested in 6 of these cases. As for the treatment results, all 3 pediatric cases who received combination chemotherapy achieved complete remission (CR). Among 6 adult cases CR was obtained in only one patient to whom low-dose cytosine arabinoside was administered. The remaining adult patients who received combination chemotherapy died relatively early.
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PMID:[Cytological features and prognosis of megakaryoblastic leukemia]. 807 88

We treated a 16-month-old girl with myelodysplastic syndrome (MDS; refractory anemia with excess of blasts subtype, RAEB by FAB classification) that developed into acute megakaryoblastic leukemia (ANLL-M7). The blast cells were positive for CD41 shown by flow cytometry and for platelet peroxidase by electron microscopy. Cytogenetically, five kinds of abnormal karyotypes were apparent at the initial visit and karyotypic progression (clonal evolution) was also evident. These karyotypes were considered to be derived from the putative original clone, 48,XX, +6, +21. The observed karyotypes were considered 50,XX, +4,add(4)(q31), +6,add(7)(p22),add(10)(q24),add(12)(q11), +20, +21, + mar[karyotype A];48,XX,add(4)(q31), +6,add(10)(q24),add(12)(q11), +21 [karyotype B];48,XX, +6,t(6;13)(p23;q14), +21 [karyotype C];51,XX, +X, t(6;13)(p23;q14), + der(6)t(6;13)(p23;q14), +21, +21, + mar [karyotype D]; and 49,XX, +X, -3,t(6;13)(p23;q14), +der(6)t(6;13)(p23;q14), -12, +21, +21, + mar [karyotype E]. It seems karyotypes B and C were derived from the putative clone; karyotype B developed into karyotype A; and karyotype C developed into karyotype E through karyotype D. After development of ANLL-M7, the cytogenetic study showed a karyotype with further karyotypic progression. The patient was treated with high-dose cytosine arabinoside (HD AraC) followed by allogeneic bone marrow transplantation. Despite intensive care, she died 3 months after the transplantation.
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PMID:Childhood myelodysplastic syndrome with clonal evolution progressing to acute megakaryoblastic leukemia (ANLL-M7). 822 7

Mutation of the ras oncogenes is the most commonly detected molecular abnormality in acute myelogenous leukemia and myelodysplastic syndromes (MDS). This molecular event may either be acquired by different subclones or by all malignant cells. The availability of the ras p21 monoclonal antibody Y13 259 makes possible the direct study of the distribution of the ras gene product in human malignant cells. The bone marrow smears from 41 patients with MDS were analysed by two independent observers after treatment with MoAb Y13 259, biotinylated goat antirat IgG, streptavidin, peroxidase and staining with diaminobenzidine. A high proportion of strongly positive smears was found among patients with MDS. This positivity was found in 25% of refractory anemia, in 80% of the refractory anemias with excess of blasts, and in 90% of those in transformation, while all 7 cases with chronic myelomonocytic leukemia were found positive. The percentage of positivity may suggest that activation of ras oncogene in associated with disease progression.
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PMID:Analysis of immunohistochemical results of the ras oncogene product p21 in myelodysplastic syndromes. 835 32

We describe a patient with primary myelodysplastic syndrome (MDS) evolving into acute nonlymphocytic leukemia (ANLL) who had two cytogenetically unrelated abnormal clones. A 68-year-old man presented with refractory anemia with excess of blasts (RAEB) and developed overt ANLL. Two cytogenetically independent clones, one with 5q- and the other with 20q-, were observed when the patient developed ANLL. The clones carrying both 5q- and 20q- were not detected. Leukemic blast cells were positive for peroxidase, naphtol ASD chloroacetate esterase, CD13, CD33, CD34 and HLA-DR, but negative for alpha-naphthyl butyrate esterase, CD14, CD10, CD19, CD20, CD1, CD2, CD3, CD5 and CD7. Although there have been a few reports describing the presence of multiple cytogenetically unrelated clones in one patient with MDS, this is the first case report that the 5q- and 20q- anomalies are derived from independent clones.
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PMID:Two karyotypically unrelated clones with 5q- and 20q- in a primary myelodysplastic syndrome patient evolving into acute nonlymphocytic leukemia. 859 Jul 73

A 27-year-old female was diagnosed as having atypical aplastic anemia in 1979 because of hypercellular bone marrow with abnormal erythroblasts and megakaryocytes. Afterward the diagnosis was corrected to myelodysplastic syndrome (RA) due to the reevaluation of the bone marrow smears. In March, 1995, thirst and polyurea occurred. In April, 1995, bone marrow aspiration biopsy showed the proliferation of atypical blasts (28%), and two months later, the number of the blasts increased (30%) and leukemic progression was noticed. Only 0.5 percent of the blasts showed weak peroxidase activity, and most of the blasts had CD13, CD33 and several adhesion molecules as CD11a, CD11b, CD44, CD54 and CD56. Karyotype of the bone marrow cells was 45, XX, -7. Her polyurea was caused by central diabetes insipidus. She was also complicated by pleuritis, colon ulcer, sinusitis and hypothalamic dysfunction. The etiology of these signs was due to the leukemic cell infiltration. She died despite of receiving multi-drug chemotherapy.
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PMID:[Acute myeloid leukemia with monosomy 7 accompanied by central diabetes insipidus]. 905 67


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