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

Erythroid and myeloid colonies were grown from the bone marrow of 81 patients with myelodysplasia and the median number of colonies correlated with the FAB classification and Bournemouth score. CFU-GM were increased in CMML compared to RAEB and RAEBt. BFU-E were higher in RA than in the other FAB subgroups. Patients with a high Bournemouth score had poorer CFU-GM and BFU-E growth than those with a low score.
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PMID:Correlation of bone marrow colony growth in the myelodysplastic syndromes with the FAB classification and the Bournemouth score. 279 88

We have examined the efficacy of various drugs in 44 patients with MDS and found the different effectiveness which depends on the type of MDS. Namely, RA appears to respond to steroid hormone, androgen, and/or vitamin D3, regardless of single or combined use. In particular, it is obvious in androgen, and as our previous reports, high content of acidic ferritin in RBC with RA have changed to more basic ones by treatment with androgen. On the contrary, these drugs were not effective on RAEB, RAEB-T, and CMML. A long-term observation is needed to determine whether the prolonged or decreased occurrence of leukemia could be obtained in the effective cases with RA. Most of the cases who did not develop overt leukemia during this study died of bleeding or infections due to thrombocytopenia or leukocytopenia, thus indicating that supportive therapies are important in patients with MDS. Since it has recently been reported that recombinant G-CSF or GM-CSF is helpful to increase the number of leucocyte and to enhance their functional recovery in MDS, these factors may be powerful agents against infections when they are carefully used with regard to the activation of leukemic clones.
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PMID:[Therapy of the preleukemic state: effect of androgens on refractory anemia]. 283 1

We followed up over a period of 10 months a Cambodian patient in whom refractory anemia with excess blasts was discovered after the onset of fever and chronic dermatologic involvement. Violaceous, firm, and painful subcutaneous nodules (1-3 cm in diameter) were present on the arms, legs, trunk, scalp, neck, and chin and were associated with violaceous infiltrating plaques on the face and forehead. The microscopic examination of repeated biopsy specimens showed a predominantly lobular panniculitis characterized by an extensive eosinophilic necrosis, leukocytoclasia, and fibrinoid deposits within a few vessels. Such lesions might be the consequence of the immune response against leukemic clones, which have been shown to be present in a steady state in at least some cases involving myelodysplastic syndromes.
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PMID:Subcutaneous eosinophilic necrosis associated with refractory anemia with an excess of myeloblasts. 291 75

We treated 11 patients who had Philadelphia-chromosome-positive chronic myelogenous leukemia with natural interferon alpha (human lymphoblastoid interferon; HLBI). HLBI was given at 6-12 X 10(6) u/day i.m. or i.s.c. during induction therapy. Nine patients responded to the treatment, of whom 7 had hematologic remission and 2 had partial remission. Six patients with MDS or hypoplastic leukemia, and 3 patients with overt leukemia from MDS were treated with recombinant interferon gamma (GI-3). GI-3 was given at 0.4 X 10(6) u/m2 of body-surface area per day i.s.c. or i.v. for 4-6 weeks. In 2 patients with RAEB and hypoplastic leukemia, the blast cell count in bone marrow decreased from 8-16% to 2-3% after 4 weeks of administration. In another patient with hypoplastic leukemia, blast cells in the marrow did not decrease, but anemia was improved without transfusion, increasing the bone marrow NCC and erythroblast count. In patients with overt leukemia and CMML, no clinical effect was obtained. Interferons can therefore be offered to patients in a preleukemic state.
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PMID:[Clinical investigation of interferons in the preleukemic state (CML and MDS)]. 313 93

Twenty patients with primary myelodysplastic syndromes (16 refractory anemia without or with ringed sideroblasts, 2 refractory anemia with excess blasts, 2 refractory anemia with excess blasts in transformation) received 13-cis-retinoic acid at a dosage of 50-100 mg/m2/day for a minimum of 4 weeks. Twelve patients obtained an increase of hemoglobin levels greater than 1 g/dl and 7 showed an associated increase of granulocyte count greater than 50% of baseline values. No significant biochemical signs of dyslipidemia or liver damage were noted. A sustained response was noted only in refractory anemia without or with ringed sideroblasts and normal or hypercellular bone marrow. Five patients are still on therapy from 23 to 82 weeks without transfusion requirement and all have shown an improvement in performance status. We conclude that 13-retinoic acid may only be clinically useful in selected patients since in myelodysplastic syndromes with blast excess the drug does not seem to improve the course of the disease.
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PMID:13-cis-Retinoic acid treatment in patients with myelodysplastic syndrome. 313 96

A low-dose Ara C (LDAC) regimen (0.2 mg/kg/d) was tried in two groups of eleven patients with MDS and in ten with hypoplastic leukemia (HL) proven by biopsy. In HL, seven patients achieved complete remission (CR), but CR duration was rather short (4-9 months). Some cases showing relapse could be induced back into CR by the LDAC regimen. All patients showed marked bone marrow aplasia before reaching CR. Therefore, the main effect of LDAC was considered to be cytotoxic activity. In one HL patient, the karyotype of 47, XX, +8 at diagnosis was converted to normal, 46, XX after CR. In MDS, there were no CR cases but five patients partially responded to the LDAC regimen. One patient died of cerebral hemorrhage soon after LDAC treatment. Three patients developed overt leukemia and in one of them the disease was well controlled by LDAC over thirty months. One patient with typical refractory anemia with excess blasts (RAEB) suffered severe bone marrow aplasia after 12 mg/d for 10 days of LDAC, but one month later his hematopoiesis gradually recovered and reached a normal hemoglobin level (12 g/dl). However, all of his karotypes showed 45, XY, -7 at that time. LDAC regimen is effective, especially for HL, but may have a limited effect on MDS.
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PMID:[Effect of low-dose Ara-C regimen on myelodysplastic syndrome (MDS)]. 316 77

Two male patients with myelodysplastic syndromes, one with refractory anemia with excess blasts (RAEB), the other with chronic myelomonocytic leukemia both had in their bone marrow and peripheral blood cells the same abnormal karyotype 46,X,-Y, + der (Y)t(Y;1)(q12;q21). This abnormality produced trisomy for the 1q21-1qter region of chromosome 1. In addition to the t(Y;1), the patient with RAEB had a del(20)(q11) abnormality in separate CFU-GM and BFUe progenitor cell populations. The t(Y;1) clone of this patient underwent chromosomal evolution with the acquisition of trisomies for chromosomes 2, 6, 8, and 9. Cytogenetic analysis of serial peripheral blood samples showed that the t(Y;1) clone and its derivatives gradually replaced that with the 20q- abnormality. Metaphase cells trisomic for chromosomes 2, 6, 8, and 9 were found predominantly in the CFU-GM population and only rarely in BFUe colonies, suggesting that chromosomal evolution was largely confined to the granulocytic lineage.
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PMID:An identical t(Y;1)(q12;q21) in two patients with myelodysplastic syndromes. 316 4

Somatic mutation of the N-ras oncogene occurs frequently in de novo acute myeloid leukemia (AML). By virtue of their relation to AML, myelodysplastic syndromes (MDS) provide an in vivo model of human leukemogenesis. By using a strategy for analysis of gene mutation based on in vitro amplification of target sequences by the polymerase chain reaction (PCR) and selective oligonucleotide hybridization we analyzed the mutational status of codons 12, 13, and 61 of Ha-ras, K-ras, and N-ras in peripheral blood (PB) and/or bone marrow (BM) in 34 cases of primary MDS. Mutations at codon 12 of Ki-ras or N-ras were detected in three cases (9%): one of six cases of refractory anemia with excess blasts (RAEB) and two of nine cases of chronic myelomonocytic leukemia (CMML). The nucleotide substitution differed in each. In all cases the mutant allele was detectable in PB cells. A sustained hematologic remission was achieved after low-dose cytarabine therapy in the case of RAEB. Neither case of CMML exhibited signs of disease progression during follow-up at 7 and 12 months. In contrast, four of 31 patients without the ras mutation underwent transformation to AML within 12 months of genetic analysis. We conclude that ras mutations in MDS are heterogeneous and may develop at an early stage during the evolution of MDS. Their detection in PB cells illustrates the potential utility of ras mutation as a clonal marker in myeloid malignancy.
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PMID:Mutation of Ki-ras and N-ras oncogenes in myelodysplastic syndromes. 328 9

Two cases of childhood myelodysplastic syndrome with chromosome abnormalities involving band 11p15 are described. The first case, with inv(11)(p15q23), had a complex clinical course; the initial diagnosis was aplastic anemia, then refractory anemia with excess of blasts in transformation (RAEB-t), and finally, before death, chronic myelomonocytic leukemia with hematologic features similar to those of chronic myelogenous leukemia (CML). The second case, with t(4;11)(p13;p15), progressed from RAEB to acute myelogenous leukemia (M2). In the literature, we found 12 patients with nonlymphocytic leukemia and chromosome abnormalities involving band 11p15, including seven cases with t(7;11)(p13-p15;p15); four cases (including the present case 1) showed CML-like hematologic features. It is suggested that translocations involving 11p15 are a nonrandom chromosome abnormality in nonlymphocytic leukemia.
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PMID:Childhood myelodysplastic syndromes with 11p15 translocation. 329 71


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