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)

A 20-year-old male developed both coccygeal and leg pain and followed by rectocystic disturbance. Disc herniation between L5 and S was suspected and laminectomy was performed. At surgery, an easily curretable tumor occupied the epidural space from L5 to the end of the sacrum. In part, the tumor spread out of the vertebral canal and invaded the surrounding muscle tissue. This muscle tissue and part of the lamina were checked histologically. Initial blood analysis revealed 5% blast-like cells, but failed to confirm them as leukemic cells. Histologically, the tumor cells had round or oval nuclei with large nucleoli and scanty cytoplasm without granulocytic differentiation. Malignant lymphoma or Ewing's sarcoma was initially suspected, but the definite diagnosis was uncertain. Immunohistochemical staining with the PAP method and enzyme histochemistry revealed that the tumor cells were positive for lysozyme and naphthol ASD chloracetate esterase. Thus, granulocytic sarcoma was finally diagnosed. Electron microscopic findings supported this diagnosis. Subsequent karyotyping of bone marrow cells revealed 8; 21 translocation, thus the final diagnosis of this patient was myelodysplastic syndrome, refractory anemia with excess blast cells in transformation or acute myelogenous leukemia, M2, by the FAB classification.
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PMID:A case of epidural granulocytic sarcoma preceding acute leukemia. 209 94

Thirty patients affected by previously untreated high risk myelodysplastic syndromes (MDS) were treated with human recombinant gamma-interferon (r-IFN-gamma): 15 of them with a higher dose (HD) of 0.1 mg/sqm, three times a week and 15 with a lower dose (LD) of 0.01 mg/sqm, three times a week, both doses administered subcutaneously (s.c.). The therapy was fairly well tolerated and few major toxic events were documented. Sustained improvement of one or more clinico-hematologic parameters was observed in 43.3% of the patients (26.6% and 60.0% for the lower and higher dose, respectively). Median survival time from the start of IFN-gamma therapy was 15+ months (range: 1-26) for patients with refractory anemia with excess of blasts (RAEB) versus 5 months (range 2-12) for patients with RAEB in transformation (RAEB-t); 15+ months (range 1-26) for HD patients versus 8 months (range 2-23) for patients treated with LD regimen; 16+ months (range 9-26) for responders versus 7 months (range 1-22) for nonresponders. All these three variables (diagnosis, treatment, and response to treatment) turned out to be statistically significant (p = at least less than 0.01) at Cox's analysis.
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PMID:Recombinant gamma-interferon as first line therapy for high risk myelodysplastic syndromes. Italian MDS Study Group. 211 13

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

Myelodysplasia, characterized by varied reductions of peripheral blood elements with normal or hypercellular bone marrow, is relatively frequent among older patients and may evolve to acute leukemia. We reviewed findings in 35 patients whom, according to the FAB classification were distributed as follows: simple refractory anemia (RA) 34%, sideroblastic refractory anemia (SRA) 14%, refractory anemia with excess blast forms (RAEB) 31%, chromic myelomonocytic leukemia (CMML) 12% and refractory anemia with excess blast forms in transformation (RAEBT) 9%. Cytogenetic studies performed in 16 patients were abnormal in 5 (31%), all among patients with poor prognosis forms of the disorder. All patients had anemia; thrombopenia and neutropenia were more frequent in subtypes RAEB, CMML and RAEBT). Mean survival rate was 30 months, significantly greater in RA and SRA compared to the other groups. Infections and development of acute leukemia were the causes of death.
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PMID:[Myelodysplasias: clinical experience with 35 patients]. 215 45

A patient with myelodysplastic syndrome (refractory anemia with excess of blasts in transformation, RAEB-T) presented with generalized granulocytic sarcomas involving the skin. The diagnosis was confirmed by skin and bone marrow biopsies. Partial myeloperoxidase deficiency was noted in the circulating polymorphonuclear leukocytes and the more differentiated tumor cells in the granulocytic sarcoma. This observation suggests that such leukocytes may be derived from the abnormal 'leukemic' clone.
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PMID:Myelodysplastic syndrome presenting with generalized cutaneous granulocytic sarcomas. 215 5

We report here a rare transformation from refractory anemia with ring sideroblasts (RARS) to chronic myelomonocytic leukemia (CMML). A rare karyotype, inv (12), was also seen at the phase of CMML. A 76-year-old female consulted a physician because of hoarseness in June, 1983. An anemia was found and blood transfusions were made. In August, 1983, she was referred and admitted to Tsukuba University Hospital for a further examination of anemia. A diagnosis of MDS (RARS) was made by hematological examinations, and pyridoxamine was administered from September, 1983. The monocyte counts in the peripheral blood increased above 1,000/microliters continuously from June, 1985, and an exacerbation of anemia was also seen. At the second admission to our hospital in August, 1988, the diagnostic criteria for CMML by the FAB co-operative group was fulfilled. At that time, chromosomal analysis revealed an abnormal karyotype; 46XY, inv (12) (p13.3 q15). Even at the phase of CMML, ringed sideroblasts were also seen in 2.2% of nucleated cell count in the bone marrow. To our knowledge, only 12 cases have been reported as transformation from another type of MDS to CMML. The present case is thought to be a rare case of transformation of MDS. On the other hand, 8 cases with inv (12) associated with malignant hematological disorders have been reported previously. Four of the above 8 cases were MDS. A relationship between development of MDS and inv (12) was suggested.
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PMID:[Chronic myelomonocytic leukemia transformed from refractory anemia with ring sideroblasts with a rare abnormal chromosome, inv (12)]. 217 2

We studied clinical and biological features of five cases of hybrid leukemia. Three of the five patients were classified as biphenotypic leukemia because of the coexpression of myeloid/B lymphoid markers in patients 1 (FAB M2) and 2 (FAB CMMoL) and myeloid/T lymphoid markers in patient 3 (FAB M4). Patient 4 was identified as bilineal-biphenotypic leukemia because acute myelogenous leukemia (AML) (FAB M4) and acute lymphoblastic leukemia (ALL) (FAB L1) coexisted and each population coexpressed myeloid and T lymphoid markers. Patient 5 was identified as bilineal leukemia due to the conversion from AML (FAB M1) to ALL (FAB L1) at an interval of 3 months. The Philadelphia (Ph1) chromosome was negative in all cases. A leukemic blast colony formation using cell line 5637 conditioned medium as a stimulator was obtained in all four patients examined. Three of the five patients had been suffering from so-called stem cell disorders such as aplastic anemia in patient 2, trilineage myelodysplasia in patient 4 and refractory anemia with excess of blasts in transformation in patient 5. The pre-existing impairment of pluripotent stem cell was probably the background of these hybrid leukemia. Hybrid leukemia appears to have an inferior prognosis: an AML-directed chemotherapy resulted in a low remission rate (2/5) with a short duration of relapse free survival (1/2) and an ALL-directed chemotherapy produced no remission (0/3). Chronological phenotypic analysis revealed that hybrid features of leukemic blasts disappeared at the time of relapse in patient 1 and progression to AML in patient 2. Monitoring of lineage-associated markers should be required for the management of hybrid leukemia.
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PMID:Clinical characteristics of hybrid leukemia: report of five cases. 217 35

To study the therapeutic effect of low-dose aclarubicin (ACR), we carried out comparative treatment of 15 patients with myelodysplastic syndrome (MDS) and atypical leukemia using this drug. Complete remission (CR) was achieved in three patients with RAEB-t and one patient with AML, partial remission was obtained in one patient with RAEB and hematological improvement in one patient with refractory anemia (RA). Interestingly, prolonged CR for more than 26 months with persistent chromosomal abnormalities was observed in a case of AML, which progressed from RA. Myelosuppression caused by low-dose ACR was milder than that caused by low-dose Ara-C. Furthermore, in vitro studies indicated that ACR induced differentiation of bone marrow cells from one patient with MDS. From these observations, it is suggested that low-dose ACR may be an alternative to low-dose Ara-C for treatment of MDS, and that the in vivo effect of ACR may be mediated by the differentiation of abnormal hemopoietic clones.
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PMID:Treatment of myelodysplastic syndrome and atypical leukemia with low-dose aclarubicin. 217 36

As part of a multicenter trial 12 patients with myelodysplastic syndromes (MDS) were treated with 14-day-cycles of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF; 250 micrograms/m2 day s.c.). In addition, all patients received 20 mg/m2/day s.c. cytosine-arabinoside (Ara-C) 12 h after GM-CSF except for patients suffering from refractory anemia (RA) according to FAB classification. Courses were repeated after 4 weeks. In 11 evaluable patients, results according to FAB-classified MDS were as follows: RA, 1/2 response (R), 1/2 stable disease (SD); RAEB, 2/3 R, 1/3 SD; RAEB-T, 1/6 CR, 1/6 PR, 2/6 R, 2/6 progression; CMML, 1/2 SD. In 2 patients with RAEB-T, overt acute myeloid leukemia was observed 2 and 10 weeks after initiation of treatment. With few exceptions, treatment resulted in a prompt increase in granulocytes and eosinophiles. This was associated with improvement of infectious complications. Increases in red cells and platelets occurred variably and was apparently associated with responses of the underlying disease. Dose limiting side effects consisted of fever, severe fatigue and dolent local reactions at the site of GM-CSF injection. In addition, nausea and diarrhoea occurred frequently. Less often, respiratory and cardiovascular side effects were encountered. In summary, GM-CSF +/- Ara-C in MDS results in objective remission with manageable toxicity. Conceivably, this regimen will serve as a base for future treatment strategies against MDS.
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PMID:Recombinant human granulocyte-macrophage colony-stimulating factor and low-dose cytosine-arabinoside in the treatment of patients with myelodysplastic syndromes. A phase II study. 218 22

Prognostic factors affecting the leukemic transformation were studied in 43 patients with myelodysplastic syndrome (MDS). Acute leukemia developed in 17 cases and it was nonlymphocytic leukemia in every case. No remission was achieved following antileukemic therapy and most of the cases proved to be true drug-resistant leukemia. Initial granulopenia, thrombopenia or anemia alone did not influence the occurrence of leukemic transformation but pancytopenia indicates bad prognosis. According to FAB classification especially refractory anemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T) were often followed by leukemic transformation. The granulocyte-macrophage progenitor cell (GM-CFC) content of bone marrow were also studied. The GM-CFC content was decreased in each patient. There was no correlation between GM-CFC number and leukemic transformation, the growth-pattern in agar-gel culture, however, turned out to have prognostic importance. Leukemic type of growth, namely always preceded leukemic transformation.
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PMID:[Factors influencing leukemic transformation in myelodysplastic syndrome]. 219 92


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