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

A retrospective clinical review of 41 patients with chronic myelomonocytic leukemia revealed a median age of 66 years and a male:female ratio of 2.4:1. The disease was preceded by a myelodysplastic syndrome of a different subtype in 24% of the patients and transformed into acute leukemia in 24%. Splenomegaly was present in 54% of the patients and reached massive proportions in 24%. Chromosomal abnormalities occurred in 34% of those studied, most commonly in the younger age group; the most frequent were trisomy 8, monosomy 7, and deletions involving the long arms of chromosomes 20 and X. Polyclonal hypergammaglobulinemia was detected in 47% of the patients in whom serum protein electrophoresis was done. The median survival was 3 years. With use of univariate analysis, the statistically significant prognostic determinants were hemoglobin level, the "modified Bournemouth score," and bone marrow blast cell percentage. When these factors were subjected to a multivariate analysis, only bone marrow blast cell percentage was an independent prognostic determinant. Orally administered hydroxyurea controlled leukocytosis and splenomegaly in some patients without affecting the overall prognosis.
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PMID:Chronic myelomonocytic leukemia: natural history and prognostic determinants. 259 15

Clinical outcome was evaluated in 43 patients with a myelodysplastic syndrome or myeloproliferative disorder and a bone marrow clone containing a single chromosome abnormality: monosomy 7/del(7q), trisomy 8, i(17q), del(5q), del(20q), or a t(2;11). Those with one of the first three abnormalities (22 patients) had shorter survival, more frequent progression to leukemia, and less response to treatment with 13-Cis-retinoic acid than did those in the latter three groups (21 patients). Additional data on these subgroups of preleukemic patients may confirm the prognostic value of such karyotypic information.
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PMID:Prognostic significance of single chromosome abnormalities in preleukemic states. 279 Jul 39

Six patients with a myelodysplastic syndrome (MDS) were treated with bone marrow transplantation (BMT) using partially-matched related (3) or unrelated (3) donors. Patients' ages ranged from 7 to 31 years (median, 10 years). Bone marrow karyotype abnormalities were present in five patients included four with monosomy 7 and one with trisomy 8. One patient was in complete remission before transplant; the remaining five had excess of blasts or were undergoing leukemic transformation. Donor, and recipient were mismatched at the DR locus (2), A locus (2), B locus (1), or A and B loci (1). Conditioning included busulfan, cytarabine, cyclophosphamide, methylprednisolone, and total body irradiation. Cyclosporine was started on day -1. Marrows were T-cell depleted using a monoclonal antibody (MoAb) (CD3) and normal rabbit serum. Four patients engrafted routinely. One patient died of aspergillosis before engraftment (day 12) and one patient failed to engraft on first attempt, but engrafted following additional preparation. Median time to neutrophils greater than 500/microL and platelets greater than 25,000/microL were 16 and 19 days, respectively. Acute graft-v-host disease (GVHD) was less than or equal to grade II in all patients. One patient died with recurrent disease (day 257). One patient died at day 515 of pancreatitis and respiratory failure. Three patients are alive and disease-free at 240, 395, and 560 days post-BMT including two patients with unrelated donors. Partially matched T-depleted bone marrow from related or unrelated donors may be effective, and possibly curative therapy for patients with MDS who lack a histocompatibility locus antigen (HLA)-identical sibling donor.
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PMID:Partially matched bone marrow transplantation in patients with myelodysplastic syndromes. 305 77

Of the myeloproliferative disorders which develop in Down's syndrome, acute leukemia associated with trisomy 8 has distinct characteristics. It is non-lymphoblastic in type and has a preleukemia phase in which thrombocytopenia is a prominent feature. One case occurring in a 20 mth-old girl is reported and 3 other similar cases reviewed. Acquired trisomy 8 in Down's syndrome is linked to leukemogenesis and confers non-lymphoblastic differentiation.
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PMID:Acute leukemia characterized by trisomy 8 in Down's syndrome. 315 66

In order to evaluate the diagnostic importance of the megakaryocytic morphology in the 5q- syndrome we studied the bone marrow from 48 unselected patients with myelodysplastic syndromes (MDS). 44 cases were primary and 4 secondary to cytostatic drug treatment or irradiation. There were 24 cases with chromosome anomalies, of whom 10 had del (5q). 4 of these had refractory anaemia (RA) with 5q- as the sole anomaly (group A), 2 had RA with 5q- and additional chromosome anomalies consisting of trisomy 8 (group B); 3 patients had RA with excess of blasts (RAEB) and complex, karyotypic changes also including 5q- (group B). Changes of the same type were found in 1 case of multiple myeloma with secondary MDS. All 6 RA patients with 5q- had characteristic megakaryocytes. More than 50% of the cells had no more than 2 nuclear segments, and predominantly had a diameter of 30 micron or more. No other patient with RA showed this picture. Only 1 patient with RAEB 5q- in group B had the same megakaryocytic changes. We conclude that diagnosis of a 5q- syndrome may be strongly suspected in cases of RA with these bone marrow changes. In cases of RAEB 5q- group B the bone marrow examination did not reveal the same consistent changes.
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PMID:Application of megakaryocytic morphology in diagnosing 5q- syndrome. 320 66

Thirteen patients with simple monosomy 7 presented with pallor in 11, easy bruisability in five, splenomegaly in four, no infections, refractory anaemia in all, granulocytopenia in seven, monocytosis in three, leucocytosis in four and thrombocytopenia in eight. Peripheral blood and bone marrow findings were consistent with myelodysplastic syndrome (MDS) type I in three, type II in two, type III in two, type IV in three and acute myelofibrosis in three patients. Transformations to acute leukaemia in seven patients were M2 in one, M4 in four, megakaryoblastic in one and undifferentiated in one. Lack of chromosome 7 in 12-85% of analysed cells at initial presentation of MDS progressed to nearly 100% during blastic transformation. At that time an additional change in the long arm of chromosome 3 was seen in two patients and trisomy 8 in 6% of analysed cells in a third case. The median survival time was 12 months for MDS and 3 months for acute leukaemia. Simple monosomy 7 appears to be largely confined to young children and elderly people.
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PMID:Simple monosomy 7 and myelodysplastic syndrome in thirteen patients without previous cytostatic treatment. 346 39

Three cases with chromosome changes involving bands 7p14 or 7p15 and 11p15 are described: one was a Japanese female with an acute myelomonocytic leukemia, the second was a white female with a 10-year history of paroxysmal nocturnal hemoglobinuria who developed a myelodysplastic syndrome, and the third was a patient with Ph-negative atypical chronic myelogenous leukemia with trisomy 8 and a chromosome change involving bands 7p14 and 11p15. These cases possibly indicate that the t(7;11)(p14 or p15;p15) change may characterize a subset of human nonlymphocytic neoplasia.
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PMID:Translocation between chromosomes 7 and 11 in nonlymphocytic neoplasia. 347 6

Ten patients with a hematologic disorder and a clone of cells with trisomy 9 in the bone marrow were studied in order to investigate the clinical significance of this chromosome anomaly. In five of the patients, trisomy 9 was the only anomaly; in four, there was also trisomy 8; and in one, a Y chromosome was also lacking. Four patients had a myelodysplastic syndrome, and six had a myeloproliferative disorder. Interestingly, four patients had primary thrombocytosis.
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PMID:Trisomy 9 in hematologic disorders: possible association with primary thrombocytosis. 347 49

Serial cytogenetic studies have been performed in four patients with myelodysplastic syndromes. In all four a 5q- alteration was present, but with a different pattern of presentation. One patient presented 5q- as the only alteration since diagnosis; two patients acquired this alteration during the course of the disease; and the fourth showed a 5q- plus other alterations since the first cytogenetic study. Likewise, three of the four patients showed a clone with trisomy 8 and without 5q-. According to these observations and others from the literature with similar cytogenetic behavior, we have analyzed the following points: 5q- as a primary event and as the only alteration, 5q- as a secondary event, 5q- plus other alterations, and presence of cytogenetically different clones. Analysis of these points suggests that the 5q- alteration can represent an early mutation conferring a slow capacity of expansion to the affected clones, with the possibility of cytogenetic evolution during the progression of the disease (about 30% of the patients). Likewise, the association of trisomy 8 clones with 5q- clones can be a nonrandom event.
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PMID:Leukemic transformation in patients with the 5q- alteration: analysis of the behavior of the 5q- clones in preleukemic to leukemic phases. 356 73

Forty-nine patients with primary myelodysplastic syndromes (MDS) were subclassified according to French-American-British (FAB) Cooperative Group criteria. Eight patients had acquired idiopathic sideroblastic anemia (AISA), ten had chronic myelomonocytic leukemia (CMMoL), 14 had refractory anemia (RA), nine had refractory anemia with excess blasts (RAEB), and five had refractory anemia with excess blasts in transformation (RAEB-T); three patients could not be subclassified. The actuarial median survival for patients with AISA or with RA had not been reached at 60 months of follow-up. The median survival times for patients with CMMoL, RAEB, and RAEB-T were 25, 21, and 16 months, respectively. The percentages of patients with each subtype who developed ANLL were none in AISA, 20% in CMMoL, 7% in RA, 56% in RAEB, and 40% in RAEB-T. Patients with CMMoL had a poor prognosis independent of transformation to acute nonlymphocytic leukemia (ANLL), whereas patients with RAEB and RAEB-T had a high incidence of transformation and short survival times. Clonal chromosomal abnormalities were present in bone marrow cells from 19 patients at the time of diagnosis, and two others developed an abnormal karyotype at the time of leukemic transformation. The most frequent abnormalities, including initial and evolutionary changes, were trisomy 8 (9 patients), deletion of 5q (4 patients), and deletion of 20q (4 patients). The median survival times were 32 months for patients with an abnormal karyotype, and 48 months for those with a normal karyotype (P = 0.2). Specific chromosomal abnormalities were not associated with particular histologic subtypes; however, a high percentage of patients with RAEB and RAEB-T had an abnormal clone (89% and 80%, respectively). The percentages of patients with clonal abnormalities were 13% for AISA, 20% for CMMoL, and 29% for RA. The MDS transformed to ANLL in 42% of patients with an abnormal karyotype, compared to 10% of those with an initially normal karyotype (P less than .01). Among patients with RA, RAEB, and RAEB-T, the risk of leukemic transformation was confined to those with an abnormal karyotype (P less than .01). Thus, in the present study, morphology and karyotype combined were the best indicators of outcome in patients with MDS.
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PMID:Prognostic implications of morphology and karyotype in primary myelodysplastic syndromes. 370 60


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