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)

Sweet's syndrome is known often to associate with non-lymphocytic leukemia (ANLL); however, there have been very few reports of Sweet's syndrome associated with myelodysplastic syndrome (MDS). It was reported that improvement and exacerbation of these two syndromes occurred simultaneously. We present here a 49-year-old male with Sweet's syndrome developed in RAEB in T. He complained of fever and infiltrative eruptions on the trunk and legs. At the time of admission to Tsukuba University Hospital, the peripheral blood showed leukocytopenia (WBC 2,000/microliter: Blast 9%, PMN 51%) and anemia (Hb 6.5 g/dl). Pseudo-Pelger anomaly of neutrophils was found on the blood smear. From the hematological findings and the result of skin biopsy, the patient was diagnosed as having MDS (RAEB in T) complicated by Sweet's syndrome. Prednisolone was effective to improve his fever and eruptions. However, when treated with low-dose Ara-C and when transformed into acute myelogenous leukemia, there was no correlation between the condition of Sweet's syndrome and the percentages of blasts in the marrow. We suggest that eruptions of Sweet's syndrome associated with MDS are not always a good index of exacerbation of MDS.
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PMID:[Appearance of Sweet's syndrome in a patient with myelodysplastic syndrome (MDS) without relation to the hematological findings of MDS]. 279 96

To determine whether cytosine arabinoside (Ara-C) has a differentiating effect in vitro, marrow cells from nine patients with acute non-lymphocytic leukemia or myelodysplastic syndrome and eight non-leukemic controls were exposed to drug concentrations comparable to those achieved in vivo with low-dose Ara-C therapy. In soft agar cultures, the predominant effect of Ara-C at concentrations between 10(-8) M and 10(-6) M was cytotoxicity with a dose-dependent decrement in Colony Forming Unit of the granulocyte and monocyte lineage (CFUg/m) at 14 days. Growth in liquid cultures containing Giant Cell Tumor(GCT)-conditioned media without Ara-C resulted in a significant increment in the recovery of mature cells at day 10 from the non-leukemic cultures (P = 0.03), while only a minor increase was found in the leukemic cultures (P = 0.09). All liquid cultures exposed to greater than or equal to 10(-9) M Ara-C showed a marked reduction in the immature proliferating cell pool, with a concomitant increase in the percentage of mature non-dividing cells at 10 days. However, the absolute number of differentiated cells recovered remained constant or decreased in all non-leukemic and eight of nine leukemic cultures, compared with cultures without Ara-C. Enhanced recovery of differentiated cells was also never observed in any culture exposed to the relatively non-toxic 10(-9) M Ara-C. These in vitro findings support clinical observations suggesting that cytotoxicity rather than differentiation is the major mechanism involved in the therapeutic effect of low-dose Ara-C in acute leukemia and myelodysplasia.
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PMID:In vitro evidence for dose-dependent cytotoxicity as the predominant effect of low dose Ara-C on human leukemic and normal marrow cells. 291 May 16

The availability of rhGM-CSF has allowed the in vivo treatment of patients with cytopenia. Therefore, a phase I-II trial was initiated to study the effect of rhGM-CSF in patients with myelodysplastic syndromes who were not eligible for other kinds of therapy. rhGM-CSF has been tested in 10 patients in doses from 15 micrograms/m2 to 150 micrograms/m2 given intravenously over 8 hours for a cycle of 7 days followed by an interval of 14 days and a second 7-day treatment course. A dose-dependent increase in leukocyte count was observed in 9 of 10 patients. No change in reticulocyte numbers was seen and only one patient experienced an increase in platelet count. Toxicity mainly consisted of mild phlebitis at the site of infusion and sternal pain after bolus injection. An increase in blast cell counts in some patients necessitated the start of low-dose Ara-C therapy.
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PMID:In vitro and in vivo action of recombinant human GM-CSF (rhGM-CSF) in patients with myelodysplastic syndromes. 306 85

The availability of rh GM-CSF has allowed the in vivo treatment of patients with cytopenia. Therefore a phase I/II trial was initiated to study the effect of rh GM-CSF in patients with myelodysplastic syndromes who were not eligible for other kinds of therapy. rh GM-CSF has been tested in 10 patients in doses from 15 micrograms/m2 to 150 micrograms/m2 given intravenously over 8 hours for a cycle of 7 days followed by an interval of 14 days and a second 7-day treatment course. A dose dependent increase in leukocyte count was observed in 9 out of 10 patients. No change in reticulocyte numbers was seen and only one patient experienced an increase in platelet count. Toxicity mainly consisted of mild local phlebitis at the site of infusion and sternal pain after bolus injection. An increase in blast cell counts in some patients necessitated the start of low dose Ara-C therapy.
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PMID:Phase I/II study with GM-CSF in patients with myelodysplastic syndromes. 307 28

Myelodysplastic syndromes (MDS) are heterogeneous diseases. Patients with blast counts of more than 20% of nucleated bone marrow cells have a high risk of short survival. We treated six patients with refractory anemia with excess of blast in transformation (RAEBiT) with low dose cytosine arabinoside (LD Ara-C). We had one partial remission (PR), surviving 16 weeks and two complete remissions (CR), surviving 22 and 55+ months. Myelosuppression was dominant in all patients, but was not as serious as with conventional remission-induction treatments for leukemias. Bone marrow aplasia occurred in all responding patients, but a differentiation effect is possible too. Maintenance therapy with LD Ara-C may be important for the two long-lasting CR.
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PMID:Low dose cytosine arabinoside in refractory anemia with excess of blasts in transformation. 320 93

Responses have been reported in patients with myelodysplastic syndromes (MDS) after low-dose cytarabine (Ara-C) or 13-cis-retinoic acid (13-CRA) therapy. Recently, combination of these two substances in vitro was shown to produce a synergistic effect on differentiation of leukemic cells. We conducted a phase II trial with low-dose Ara-C (5 mg/m2 per 12 h s.c.) and 13-CRA (60 mg/m2 per day orally) in 14 patients with MDS, six of whom had refractory anemia with excess of blasts (RAEB), seven had RAEB in transformation (RAEBt) and one chronic myelomonocytic leukemia (CMML). The drugs were administered from day 1 to 14 and the treatment courses repeated every 4 to 8 weeks. One partial response and one minor response could be achieved. Major toxicity included dry skin, mucositis and cheilitis in 11 of the 14 patients. The response rate is no better than the results reported in the literature with either drugs alone. As yet there is no satisfactory treatment for MDS.
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PMID:Combination of low-dose cytarabine and 13-cis retinoic acid in the treatment of myelodysplastic syndromes. 332 May 77

A small group of elderly male patients received attenuated doses of daunomycin, cytosine arabinoside (Ara-C), and 6-thioguanine for treatment of myelodysplastic syndrome (MDS). Three patients had refractory anemia with excess blasts (RAEB), and two had chronic myelomonocytic leukemia (CMMoL). All five patients had developed severe transfusion requirements for platelets and red blood cells before therapy was begun. One patient developed necrosis of the cecum and expired 19 days after therapy, but the other four all showed substantial benefit from treatment. Three of those patients received multiple courses of chemotherapy which led to improvement in peripheral blood counts in each case. Duration of responses as noted by improvement in peripheral blood counts compared to pretreatment levels ranged from 1.5 to 9 months. Despite considerable improvement in peripheral blood parameters, some of the abnormal morphologic features of MDS persisted after each course of chemotherapy. These results obtained with attenuated chemotherapy schedules in a small group of patients are sufficiently encouraging to warrant an expanded phase II trial, which is under way at the University of Rochester Cancer Center.
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PMID:Effectiveness of attenuated chemotherapy in myelodysplastic syndromes: a preliminary report. 335 33

62 evaluable patients with myelodysplastic syndromes (MDS) or acute leukemia were treated with different combinations of low dose ara-C, alpha-interferon (IFN), 1 alpha-hydroxyvitamin D3 (vit D3) and retinoic acid. The aim was to study the efficacy and toxicity of each combination. The overall rate was 44%. Of these, 50% responded favorably to the combination of IFN, vit D3 and retinoic acid (IDR), which was comparable to the response rate of 43% for low-dose ara-C. The results of the IDR treatment may be explained by additive or synergistic effects between the separate drugs in the combination. Ara-C and IDR treatment was generally well-tolerated but interferon gave more side effects than any other drug used in the study. Evaluation of the full combination of ara-C, IFN, vit D3 and retinoic acid was not possible because of toxicity. Marrow hypoplasia was infrequent (5/27 patients) in cases responding favorably to treatment. Complete remissions were not longer than partial remissions or significant responses.
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PMID:Therapeutic effects of low-dose cytosine arabinoside, alpha-interferon, 1 alpha-hydroxyvitamin D3 and retinoic acid in acute leukemia and myelodysplastic syndromes. 337 98

This study was designed to evaluate the effect of low-dose Ara-C in the treatment of refractory, atypical leukemia and myelodysplastic syndrome (MDS). The subjects were 33 patients (19 acute myelocytic leukemia (AML), 9 MDS, 3 atypical leukemia and 2 unclassified leukemia). The age range was 19 to 84 years with a mean age of 51.5 years. We administered low-dose Ara-C (5-10 mg/m2/12 h s.c. or i.v.) for 13 to 35 days with a mean of 16.7 days. Complete remission and partial remission were obtained in 8 of 31 (25.8%) and in 9 of 31 (29.0%) respectively. A high response rate was found in M2 types showing a tendency for mature and refractory anemia with excess blasts in MDS according to the FAB classification. In our cases, severe pancytopenia in peripheral blood as a result of Ara-C was observed before normal hematopoiesis recurred. From the above results, the mechanism of low-dose Ara-C may induce remission by a cytotoxic rather than a differentiation effect. This treatment can be offered to patients with refractory, atypical leukemia and MDS with limited toxicity.
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PMID:[Effect of low-dose Ara-C in the treatment of refractory, atypical leukemia and myelodysplastic syndrome]. 345 53

The bone marrow smears and marrow biopsies of 30 unselected consecutive patients with a myelodysplastic syndrome have been classified retrospectively according to the proposals of the French-American-British cooperative group (FAB). The diagnoses according to the FAB classification were refractory anemia (RA) in 3, RA with ring sideroblasts (RAS) in 5, RA with excess of blasts (RAEB) in 14, REAB in transformation (RAEB-t) in 5, and chronic myelomonocytic leukemia (CMML) in 3. The group comprised 19 men and 11 women with a median age of 68 years. Of 22 patients, 9 died after progression to acute non lymphoblastic leukemia (ANLL), 7 from infections, 2 from bleeding and 4 from unrelated causes. Actuarial survival is 10.5 months, 8 patients being at risk at the time of evaluation. Although the small number of patients does not allow statistical evaluation, our results are similar to those in the literature: prognosis is less good with an increasing number of marrow blasts and degree of cytopenia, and the risk of progression to ANLL is higher for REAB-t and REAB patients. Of 4 patients treated with small doses of cytosine-arabinoside (Ara-C), one responded and remained stable without treatment for 7 months. 2 patients resistant to small-dose Ara-C showed complete response to subsequent high-dose Ara-C treatment.
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PMID:[Retrospective study of 30 cases of myelodysplastic syndrome according to the French-American-British classification]. 346 10


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