1/11. Importance of basophilia in haematopoietic disorders.To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17).- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
2/11. patients with myelodysplastic syndromes benefit from palliative therapy with amifostine, pentoxifylline, and ciprofloxacin with or without dexamethasone.Thirty-five patients with myelodysplastic syndrome (MDS) were registered on protocol MDS 96-02 and were receiving continuous therapy with pentoxifylline 800 mg 3 times a day and ciprofloxacin 500 mg twice a day by mouth; dexamethasone was added to the regimen for the partial responders and the nonresponders after 12 weeks at a dose of 4 mg by mouth every morning for 4 weeks. amifostine was administered intravenously 3 times a week at 3 dose levels (200 mg/M(2), 300 mg/M(2), and 400 mg/M(2)) to cohorts of 10 patients each. Therapy has been continued for 1 year in responders. Twenty-nine have completed at least 12 weeks of therapy and are available for response evaluation. Of the 21 men and 8 women (median age, 67 years), 20 had refractory anemia (RA), 3 had RA with ringed sideroblasts (RARS), 5 had RA with excess blasts (RAEB), and 1 had chronic myelomonocytic leukemia (CMMoL). Five had secondary MDS. No differences were noted in response rates among the 3 dose levels. Seven patients did not respond at all, and 22 showed an improvement in cytopenias (76%). Three had a triple lineage response, 10 had a double lineage response, and 9 had a single lineage response (8 of 9 in absolute neutrophil count [ANC] and 1 had more than a 50% reduction in packed red blood cell transfusions). Fifteen patients responded only after the addition of dexamethasone, whereas 7 responded before. When examined by lineage, 19 of 22 showed improved ANC, 11 of 22 demonstrated more than 50% reduction in blood transfusions, improved Hb levels, or both, and 7 of 22 showed improvement in platelet counts. Interestingly, the responses were frequently slow to appear, and continued improvement in counts was seen up to 12 months of therapy and beyond. This study supports the feasibility of treating patients with MDS with the unique approach of cytoprotection and anticytokine therapies as well as the principle that prolonged commitment to treatment is desirable when noncytotoxic agents are administered. (blood. 2000;95:1580-1587)- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
3/11. Derivative (1;18)(q10;q10): a recurrent and novel unbalanced translocation involving 1q in myeloid disorders.We report two cases of hematological malignancies, comprising a case of myelodysplastic syndrome (MDS) that rapidly evolved into acute myeloid leukemia, and a case of myeloproliferative disorder (MPD), in which der(1;18)(q10;q10) was found as the sole acquired karyotypic abnormality. This observation indicates that the unbalanced translocation is a recurrent aberration in myeloid disorders. To the best of our knowledge, centromeric fusion between long arms of chromosomes 1 and 18, leading to a normal chromosome 18 substituted with a der(1;18) chromosome, is novel and has not been described in cancer. Mechanistically, either trisomy 1q or monosomy 18p that results from the translocation may potentially contribute to leukemogenesis. Finally, chromosomes with large constitutive heterochromatin bands such as chromosome 1 may be at risk of centromeric instability and be predisposed to centromeric fusion with other chromosomes.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
4/11. pyomyositis as a focus of infection in hematological disorders: a report of 3 cases.The cases of 3 patients with pyomyositis associated with hematological disorders are reported. A 40-year-old man in the blastic phase of chronic myelogenous leukemia and 2 men aged 46 and 71 years with neutropenia due to myelodysplastic syndromes all reported high fever and severe local myalgia and had marked elevation of c-reactive protein. magnetic resonance imaging revealed muscle abscesses or fasciitis, and the findings led to the diagnosis of pyomyositis. methicillin-resistant staphylococcus aureus was isolated from the abscesses of 2 patients, and surgical drainage proved more effective than did antimicrobial agents. It should be recognized that pyomyositis is a possible source of infection in patients with hematological disorders.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
5/11. Chromosome 6p rearrangements appear to be secondary changes in various haematological malignancies.We report on six cases of 6p rearrangement in various haematological malignancies. On reviewing the literature, we assume 6p rearrangements to be secondary anomalies in both myeloid and lymphoid malignancies, and confirm it to be strongly associated with -5/del (5q) in myelodysplastic syndromes.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
6/11. Simultaneous expression of lymphoid and myeloid phenotypes in acute leukemia arising from myelodysplastic syndrome.A 57-year-old female developed myelodysplastic syndrome (MDS) that terminated as a biphenotypic leukemia after exposure to chemoradiotherapy. Double staining of blast cells, using monoclonal antibodies specific for myeloid and lymphoid lineage, demonstrated that one-third of the leukemic cells simultaneously expressed the E rosette-associated antigen (OKT11) and myeloid-associated antigen (MY7). This finding suggests the possibility that some cases of MDS are clonal disorders that arise in a pluripotent stem cell that can also differentiate to T cell lineage.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
7/11. Major karyotype aberrations, including t(3;12), in a patient with myelodysplastic syndrome.cytogenetic analysis of bone marrow cells was performed twice on a patient with myelodysplastic syndrome, specifically, refractory anemia with excess blasts. The patient had a progressive course, with transformation toward acute leukemia and death within 3 months. Chromosome analysis showed major karyotype abnormalities, including dir dup (1p), t(3;12), and a unique breakage of a #15 resulting in t(15;18) and dic(15;21). Involvement of #3 and #12 in a translocation has been recently reported, and a comparison with these cases is made with a discussion of the significance.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
8/11. A patient with Tn syndrome associated with myelodysplastic syndrome showed abnormal glycophorin B.A Japanese male patient with myelodysplastic syndrome (MDS) was shown to have associated Tn syndrome; the first report of Tn syndrome with MDS. The Tn expression was demonstrated on erythrocytes, granulocytes, monocytes, platelets, and lymphocytes by flow cytometric analysis using a lectin and an antibody. electrophoresis of erythrocyte membrane proteins revealed slower mobility of glycophorin B from the patient than that from normal individuals, suggesting a glycophorin B molecular abnormality.- - - - - - - - - - ranking = 5keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
9/11. etoposide and secondary haematological malignancies: coincidence or causality?BACKGROUND: Secondary haematological malignancies (SHM) induced by alkylating agents show cytogenetic abnormalities involving mainly chromosomes 5 and 7. etoposide and other topoisomerase ii inhibitors including anthracyclines induce secondary leukaemias with a short latency period, briefly preceded by a myelodysplastic phase, and frequently associated with balanced translocation characteristically involving the long arms of chromosomes 11 and 21. patients AND methods: etoposide with doxorubicin containing chemotherapy was administered to 26 patients with Hodgkin's disease (HD), eight of whom received alkylating agents at relapse or progression, and to 59 patients with small-cell lung carcinoma (SCLC), thirty-three of whom received alkylating agents in the same combination. RESULTS: Four of 85 patients developed SHM of the myelodysplastic or acute myeloid type at, respectively, 20, 30, 35 and 38 months after therapy. In 3 of them, chromosome abnormalities were observed: two balanced translocations, t(6;9)(p23;34) and t(11;19)(q23;p13), and one monosomy 7q due to t(1;7)(cen;cen) with trisomy 8. The cumulative risk estimate of SHM is 12% (95% confidence interval (CI): 3%-46%) at 5 years for patients with HD and 18% (95% CI: 5%-49%) at 3 years for patients with SCLC. CONCLUSIONS: Our observations lend further support to the existence of SHM induced by topoisomerase ii inhibitors that present early after initial treatment with balanced translocations to 11q23 and 21q22. However, other defects such as unbalanced abnormalities of chromosomes 5 and 7 can occur and may be related to the combination of topoisomerase ii inhibitors with alkylating agents. Balanced t(6;9) was observed for the first time in SHM. A synergism between epipodophyllotoxins, anthracyclines, alkylating agents, cisplatin and radiotherapy is suggested, given the observed high risk of SHM.- - - - - - - - - - ranking = 0.56583919271783keywords = myelodysplastic (Clic here for more details about this article) |
10/11. Hematological abnormalities and cholestatic liver disease in two patients with mevalonate kinase deficiency.We describe two patients with mevalonate kinase deficiency and prominent hematologic abnormalities and cholestatic liver disease. Patient R.B. was not anemic at birth, but developed petechiae and cutaneous extramedullary hematopoiesis, hepatosplenomegaly, leukocytosis, and recurrent febrile events without positive bacterial or viral cultures. Patient N.M. manifested minor anomalies, hepatosplenomegaly, anemia, thrombocytopenia, recurrent febrile crises, and facial rashes. Mevalonic aciduria was found by urinary organic acid analysis, and mevalonate kinase deficiency was documented in both. The clinical spectrum of normocytic hypoplastic anemia, leukocytosis, thrombocytopenia, and abnormal blood cell forms led to diagnoses of congenital infection, myelodysplastic syndromes, or chronic leukemia in these patients before recognition of mevalonate kinase deficiency. mevalonate kinase deficiency represents a single-gene abnormality that may be associated with significant hematologic findings. Recognition of the variability of this disorder with some patients manifesting only mild neurologic findings, yet significant hepatosplenomegaly, normocytic anemia, thrombocytopenia, and leukocytosis is important for all specialists who need to be aware of this organic aciduria.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
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