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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

If chronic myelomonocytic leukemia (CMML) transforms into an acute leukemic phase, the blast crisis is invariably myeloid. Occasionally, the other subtypes of myelodysplastic syndrome (MDS) (refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation) have been noted to transform into acute lymphoblastic leukemia (ALL). We now report a case of CMML that transformed into ALL and we review the literature of 13 other cases of MDS with ALL transformation. Such cases provide suggestive clinical evidence that MDS can involve a pluripotent stem cell.
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PMID:Transformation of chronic myelomonocytic leukemia to acute lymphoblastic leukemia: case report and review of the literature of lymphoblastic transformation of myelodysplastic syndrome. 777 69

The aim of this study was to evaluate the possible prognostic relevance of thymidine kinase serum levels (s-TK), an indirect marker of proliferative activity, in myelodysplastic syndromes (MDS). S-TK levels were monitored by means of a radioenzyme assay in 90 patients affected by MDS (22 refractory anaemia, RA; 17 RA with ring sideroblasts, RARS; 21 RA with blast excess, RAEB; 15 RAEB in transformation, RAEB-T; 15 chronic myelomonocytic leukaemia, CMMoL). Mean s-TK levels (U/microliter) measured at diagnosis were 11.9 +/- 12.6 for RA, 11.4 +/- 13.6 for RARS, 19.9 +/- 28.4 for RAEB, 39.6 +/- 34.3 for RAEB-T and 77.7 +/- 69.7 for CMMoL (normal values < 5 U/microliter). With the only exception of a weak relationship with lactate dehydrogenase, no correlation was found between initial s-TK values and other clinical or laboratory parameters, such as age, haemoglobin, white blood cell or platelet count, percentage of bone marrow blasts. MDS patients with s-TK > 38 U/microliters, a cut-off level selected by means of ROC statistical analysis, showed a significantly shorter survival than those with s-TK < 38 U/microliter (8.2 v 37.4 months, respectively; P < 0.0001). In particular, transformation in acute myeloid leukaemia (AML) occurred in 17/21 (81%) of patients with s-TK > 38 U/microliters and 9/69 (13%) of those with lower levels at diagnosis (P < 0.0001), independently of FAB subtype. High s-TK levels were also useful to predict evolution in AML during the course of the disease in patients with normal initial values. Multivariate analysis confirmed the independent prognostic value of s-TK on both overall survival and risk of acute transformation. We conclude that s-TK may be an important prognostic factor in MDS, strongly correlated with development of AML.
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PMID:Prognostic relevance of serum thymidine kinase in primary myelodysplastic syndromes: relationship to development of acute myeloid leukaemia. 778 74

20 cases of myelodysplastic syndrome (MDS) patients were treated with Yisuikang (YSK) including MDS-RA 17 cases, MDS-RAEB 2 cases, MDS-RAEB-T 1 case. 14 patients were treated with YSK only, 6 cases were treated with both YSK and Western medicine. The result showed that 4 cases were remitted, 5 cases markedly improved, 6 cases improved and 5 cases ineffective, the total effective rate was 75.0%. It was found that YSK was effective in the treatment of MDS-RA or Qi-Blood Deficiency and Yu-Re Syndrome in TCM, while any other types were ineffective. They also found that types of TCM might be correlated with MDS.
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PMID:[Clinical observation on treatment of myelodysplastic syndromes with yisuikang]. 778 94

The myelodysplastic syndromes are a group of hematologic disorders that adversely affect the levels of hemoglobin, platelets, erythrocytes, and leukocytes. Although the cause of this syndrome is unknown, new diagnostic techniques have facilitated identification and classification of these diseases into five categories: refractory anemia (refractory cytopenia), refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and chronic myelomonocytic leukemia. Cytogenetic abnormalities may be present in more than 55% of the patients. Symptomatic patients should be assessed relative to life-threatening versus non-life-threatening cytopenias and age. Management consists of primarily supportive measures, although certain approaches that are currently being used or under investigation, such as concomitant administration of erythropoietin and other growth factors, show promise for the future.
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PMID:Myelodysplastic (preleukemia) syndromes: the bone marrow factory failure problem. 779 91

Myelodysplastic syndromes (MDS) represent an acquired group of clonal disorders of the pleuripotent stem cells, resulting in progressive life-threatening cytopenias or transformation into acute leukemias. A major issue of using alloBMT in MDS is the criteria used for patient selection. Therapeutic trials of lesser intensity such as differentiating agents, and cytokines could be the preferable choice for patients with good prognostic features. On the other hand, patients with poor prognostic features may urgently need to establish a normal hematopoiesis through allogeneic bone marrow transplantation (alloBMT). Important prognostic indicators in MDS include FAB classification, presence of abnormal localization of immature precursors, degree of cytopenias and cytogenetic abnormalities. We used a novel preparative regimen--"BAC" consisting of the consecutive administration of 1 mg/kg of busulfan every 6 hours for 16 doses followed by 2 g/M2 of cytosine arabinoside (ara-C) given every 12 hours for four doses, and finally 60 mg/kg of cyclophosphamide daily for 2 days. Thirty two patients transplanted had a median age of 33 years. Nine of the patients had either RA or RARS, 21 had RAEB or RAEB-T and 2 were unclassified MDS. Twenty two of our patients had chromosomal abnormalities while 10 had a normal karyotype analysis. Nine of the 32 patients had documented leukemic transformation and received induction therapy prior to BMT. The median time from diagnosis to BMT was 5.6 months, ranging from 1.3 to 30.2 months. Nineteen out of 32 patients are alive without disease, with a median follow up of 24 months. The actuarial event-free survival for the entire group is 52%. Two patients have relapsed with an actuarial relapse rate of 12%. Only significant favorable prognostic indicator for the event-free survival was in the recipient of a genotypically matched graft (76%) compared to recipients of a non-genotypic graft (23%) (p = 0.02). "BAC" is a unique preparative regimen for alloBMT in MDS with excellent results.
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PMID:Allogeneic bone marrow transplantation in patients with myelodysplastic syndromes. 781 96

A systematic morphological analysis of cutaneous infiltrates in acute myelogenous leukemia and myelodysplastic syndrome revealed that in many cases the infiltrating cells have a different phenotype from those in the bone marrow. This study sought to answer two questions: (a) How wide is the range of cytological features and immunoreactivity of the cutaneous infiltrates and what danger is there of misinterpretation? (b) What are the possible causes of the wide spectrum of differentiation of the cells infiltrating the skin? Skin biopsy specimens from 16 patients with myelogenous leukemia or myelodysplastic syndrome were investigated. The diagnosis was acute myelomonocytic leukemia (M4, according to the French-American-British/FAB system of classification of acute leukemias) in eight cases, acute monocytic leukemia (M5) in four cases, aleukemic leukemia cutis as a recurrence of M2 leukemia after treatment in one case, and myelodysplastic syndrome in three cases, including one case of myelodysplasia with an excess of bone marrow blasts (RAEB-T) and two cases of chronic myelomonocytic leukemia, one of which presented as aleukemic leukemia cutis. Reactivity with the macrophage-associated antibodies anti-CD68, Ki-M1p, and anti-lysozyme was the most consistent. However, the naphthol AS-D chloroacetate esterase reaction and staining with DAKO-M1, Ki-My2p, anti-neutrophil elastase, and anti-CD34 were found to be of little value for identifying the cutaneous infiltrate as myelogenous. Some antibodies (e.g., anti-S100 protein and MB2) even produced staining in a few cases that could have led to a mistaken diagnosis of histiocytic neoplasm or malignant lymphoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Skin involvement in myelogenous leukemia: morphologic and immunophenotypic heterogeneity of skin infiltrates. 754 88

The clinical data and hematological features of 29 children, under the age of 12 years, with primary myelodysplasia are presented. The diagnosis was made using the FAB (French-American-British) Cooperative Group criteria. There were 24 males and 5 females aged 4 months to 12 years (median 2.5 years) with marked male preponderance. Childhood myelodysplasia constituted 16% of all hematological malignancies and 36.7% of acute myeloid leukemias. The median duration of symptoms prior to diagnosis was 3 months. There were 15 cases of refractory anemia, one of refractory anemia with excess blasts, 3 of refractory anemia with excess blasts in transformation and 10 cases of chronic myelomonocytic leukemia. Five patients evolved to acute myeloid and 4 to acute lymphatic leukemia. The median duration of preleukemic phase in these patients was 7 months (range 4-29 months). The overall mean survival was short (5-9 months) in all the subgroups. Besides supportive therapy in most patients, two patients were treated with etoposide, one with alfa interferon 2b and one with high dose methylprednisolone. Our results show that myelodysplasia is not infrequent in children. The disease has an aggressive clinical course and may evolve into acute leukemia.
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PMID:Childhood myelodysplasia. 789 Mar 42

The EVI1 gene encodes a zinc-finger, DNA-binding protein originally described as the transforming gene associated with a common ecotropic viral insertion site in myeloid leukemias. Previous studies demonstrated EVI1 expression in human leukemias in cases with 3q26 translocations, but not in normal blood or bone marrow. These studies also suggested an association between EVI1 expression and chromosome 7 deletion (del). Because of this association, we examined expression of EVI1 using RNA polymerase chain reaction (PCR) in patients with myelodysplastic syndromes (MDS) and acute leukemia with and without 3q26 translocations. EVI1 RNA was expressed in 29% of 34 (95% confidence interval, 20% to 50%) patients with the MDS subtypes refractory anemia (RA), refractory anemia with excess blasts (RAEB), or refractory anemia with excess blasts in transformation (RAEB-T). The vast majority of these cases occurred in patients with RAEB and RAEB-T. EVI1 expression was not detected in patients with chronic myelomonocytic leukemia (CMML), normal bone marrow or cord blood, or a variety of other hematologic malignancies. EVI1 RNA was detected in three of 18 patients with acute myelogenous leukemia (AML) and in two of four patients with acute promyelocytic leukemia (APL). Karyotypes showed that only one AML patient had karyotype 3q26 abnormalities, indicating that EVI1 expression is associated with cases that do not have structural abnormalities involving chromosome 3q26. These studies document for the first time the abnormal expression of EVI1 RNA by patients with MDS, and suggest an important role for EVI1 in the pathogenesis or progression of some myeloid malignancies.
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PMID:Expression of EVI1 in myelodysplastic syndromes and other hematologic malignancies without 3q26 translocations. 804 40

The therapy for myelodysplastic syndromes (MDS) and acute leukemia (AL) transformed from MDS is not well established. Etoposide (VP 16-213) at low concentrations shows differentiation-inducing activity against leukemic cells in vitro. A prior study showed that oral low-dose etoposide therapy was effective in patients with chronic myelomonocytic leukemia. We used low-dose etoposide to treat six patients with refractory anemia with excess blasts in transformation (RAEB-t) and seven patients with AL transformed from MDS. The etoposide (50 mg, 2-7 times/week) was usually administered intravenously to ensure reliable bioavailability. Of 12 assessable patients, four RAEB-t patients achieved a partial response and one AL patient achieved complete remission. The responders became transfusion-independent, and this continued for 2-9 months while etoposide therapy was continued. Three of five responders had been resistant to prior repeated low-dose cytarabine therapy. The side effects were mild and well tolerated. Heterogeneous mechanisms were surmised to explain the clinical effects of low-dose etoposide. Several aspects, including the optimal schedule of low-dose etoposide therapy, the effect of this therapy on the patients' survival, the usefulness of combination therapy with other chemotherapeutic drug(s) and/or cytokine(s), should be investigated in the future.
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PMID:Application of low-dose etoposide therapy for myelodysplastic syndromes. 816 35

Interstitial loss of the long arm of chromosome 5 (5q-) is an anomaly frequently seen in myelodysplasia (MDS) and acute myelogenous leukemia (AML). Although the limits of the interstitial deletions vary among patients, there is a critical region of overlap at 5q31 that is consistently deleted in most cases. The order of genes in the critical 5q31 region is centromere, interleukin gene cluster, an anonymous polymorphic locus D5S89, early growth response factor, CSF1 receptor, telomere. Fluorescence in situ hybridization of specific 5q31 probes to metaphases with del(5) (q11q31) from a patient with secondary refractory anemia with excess blasts in transformation demonstrates that the interstitial deletion is not contiguous. The 5q- chromosome has lost the D5S89 and CSF1R loci while retaining some of the sequences in between. A probe derived from a 300-kbp yeast artificial chromosome containing the D5S89 locus is interrupted on the normal chromosome 5 of this patient. Data presented in this report are consistent with (i) presence of a critical gene within the YAC and (ii) more than a single interstitial break within the 5q- chromosome. These results, while pinpointing one of the critical 5q31 loci, also provide evidence for a second telomeric locus.
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PMID:5q- chromosome. Evidence for complex interstitial breaks in a case of refractory anemia with excess blasts. 819 54


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