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

We describe a novel continuous B-cell line (PV-90) derived from a patient with myelodysplastic syndrome (MDS) and originating from spontaneous infection with the Epstein-Barr virus (EBV). The patient progressed to acute myeloblastic leukaemia (AML) 5 months after clinical onset of MDS. PV-90 is of clonal origin as indicated by the presence of immunoglobulin (Ig) gene rearrangements, monoclonal surface immunoglobulins, and a single DNA restriction fragment corresponding to the EBV genomic termini. PV-90 cells also express a number of myelomonocytic markers, including alpha-naphthyl acetate esterase (ANAE), coagulation factor XIII, and CD68 antigen. Moreover, PV-90 cells constitutively express the c-fms proto-oncogene mRNA as the patient's blast cells did. Whereas a trisomy 11 (+11) was found in the patient's bone marrow cells, PV-90 cells had a normal karyotype initially, but at 4 months showed two different and independent chromosomal abnormalities: 90, XX, -Y, -Y, t(9;16) (q11;p13), and 90, XX, -Y, -Y, t(17;18) (p13;q21), the latter possibly involving the p53 (17,p13) and bcl-2 (18, q21) proto-oncogenes. The early development of these chromosomal aberrations is consistent with a genetic instability of PV-90 cells. Expression of bi-lineage markers and genetic instability may suggest that PV-90 cells originated from transformation of a myelodysplastic progenitor cell capable of both myeloid and B-cell differentiation. The PV-90 cell line might be useful in a number of studies, including the possible role of c-fms in cell differentiation, pathogenetic mechanisms of human preleukaemia and lineage promiscuity in acute leukaemia.
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PMID:Establishment and characterization of a B-cell line derived from a patient with a myelodysplastic syndrome which expresses myelomonocytic and lymphoid markers. 164 72

Oral hairy leukoplakia (OHL) has been observed in all risk groups seropositive for HIV infection. Recently, this lesion has also been described in HIV-seronegative patients with immunosuppression of iatrogenic origin. We report on a HIV-1 and HIV-2 seronegative, heterosexual man affected by refractory anemia with ringed sideroblasts (myelodysplastic syndrome), who developed recurrent oral condylomata acuminata and OHL as an early clinical manifestation. The diagnosis of OHL was confirmed by identifying Epstein-Barr viral particles by electron microscopy and by in situ DNA hybridization. HIV infection was ruled out using polymerase chain reaction and testing for HIV-1 and HIV-2 antibodies.
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PMID:Recurrent oral condylomata acuminata and hairy leukoplakia: an early sign of myelodysplastic syndrome in an HIV-seronegative patient. 165 14

Seventy-one patients with hematologic malignancies received bone marrow from a histocompatible sibling (n = 48) or a partially matched relative (n = 23) that had been depleted of CD5+ T cells with either an anti-CD5 mooclonal antibody (MoAb) plus complement (anti-Leu1 + C) or an anti-CD5 MoAb conjugated to ricin A chain (ST1 immunotoxin [ST1-IT]). These patients received intensive chemoradiotherapy consisting of cytosine arabinoside, cyclophosphamide, and fractionated total body irradiation. Both anti-Leu1 + C and ST1-IT ex vivo treatments effectively depleted bone marrow of T cells (97% and 95%, respectively). Overall, primary and late graft failure each occurred in 4% of evaluable patients. The diagnosis of myelodysplasia was a significant risk factor for graft failure (P less than .001), and if myelodysplastic patients were excluded, there were no graft failures in major histocompatibility complex (MHC)-matched patients and 2 of 23 (8.7%) in MHC-mismatched patients. The actuarial risk of grade 2 to 4 acute graft-versus-host disease (GVHD) was 23% in MHC-matched patients and 50% in MHC-mismatched patients. In MHC-matched patients, acute GVHD tended to be mild and treatable with corticosteroids. Chronic GVHD was observed in 6 of 36 (17%) MHC-matched patients and none of 11 MHC-mismatched patients. There were no deaths attributable to GVHD in the MHC-matched group. Epstein-Barr virus-associated lymphoproliferative disorders were observed in 3 of 23 MHC-mismatched patients. The actuarial event-free survival was 38% in the MHC-matched patients versus 21% in the MHC-mismatched patients. However, if outcome is analyzed by risk of relapse, low-risk patients had a 62% actuarial survival compared with 11% in high-risk patients. These data indicate that the use of anti-CD5 MoAbs can effectively control GVHD in histocompatible patients, and that additional strategies are required in MHC-mismatched and high-risk patients.
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PMID:Selective depletion of bone marrow T lymphocytes with anti-CD5 monoclonal antibodies: effective prophylaxis for graft-versus-host disease in patients with hematologic malignancies. 171 80

A case of therapy-related myelodysplasia followed by acute nonlymphocytic leukemia in a 5-year-old child successfully treated for diffuse Langerhans cell histiocytosis is described. A syndrome of severe cell-mediated immune deficiency and persistent Epstein-Barr virus (EBV) infection coincided with the evolution of myelodysplasia. Specific abnormalities of chromosomes number 7 and 3 were associated with the onset of myelodysplasia and acute nonlymphocytic leukemia and are believed to be linked to the patient's immune dysfunction and compromised ability to contain viral infection.
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PMID:Acquired immune deficiency, myelodysplasia, and acute nonlymphocytic leukemia associated with monosomy 7 and t(3;3) (q21;q26) in a child with Langerhans cell histiocytosis. 254 62

A new hematopoietic cell line derived from a patient with Philadelphia chromosome (Ph1)-negative myeloblastic leukemia arising from a form of myelodysplastic syndrome (MDS) is described. This cell line, designated TMM, consists of immature cells with the morphological characteristics of young myeloblasts and grows in suspension culture with a doubling time of about 30 hours. By cytochemical analysis the cultured cells were positive for acid phosphatase. They were free of the Epstein-Barr virus-associated nuclear antigen as well as terminal deoxynucleotidyl transferase. Further phenotypic analysis revealed the expression of the myelomonocytic-specific antigen Leu-M1 and receptors for the Fc portion of IgG. Partial differentiation of these cells could be induced by dimethyl sulfoxide, tetradecanoyl phorbol acetate, or hypoxanthine and resulted in cells of the myeloid series expressing lysozyme and receptors for the C3b complement protein. The karyotype was 46,XY, lacked the Ph1 chromosome, and displayed no abnormalities at the light microscopic level. No rearrangement of the bcr-c-abl gene complex was found. This cell line should be useful for studying an important type of the heterogeneous population constituting Ph1-negative myeloblastic leukemia, arising in this instance from MDS, as well as for studying differentiation and proliferation of human pluripotent stem cells.
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PMID:Establishment and characterization of a human myeloid cell line from Philadelphia chromosome-negative myeloblastic leukemia arising in a patient with myelodysplastic syndrome. 347 6

Somatic cell genetic approaches utilizing the cellular mosaicism present in women heterozygous for glucose-6-phosphate dehydrogenase (G6PD) have provided information relevant to the pathogenesis of some neoplastic disorders. With these techniques, we studied a 61-year-old woman with a myelodysplastic syndrome. GdB/GdA heterozygosity was demonstrated in skin and cultured T lymphocytes, which exhibited both A and B type G6PD. In contrast, erythrocytes, platelets, granulocytes, and marrow nucleated cells displayed almost exclusively G6PD type B. In addition, 21 of 24 Epstein-Barr virus-transformed B lymphoblastoid lines that expressed a single immunoglobulin light chain showed only type B G6PD, suggesting that the stem cells involved by this disease were clonal and could differentiate to B lymphocytes as well as to mature granulocytes, erythrocytes , and platelets. Cultured skin fibroblasts and phytohemagglutinin-stimulated lymphocytes were karyotypically normal, but two independent abnormalities were found in marrow--47,XX, +8 and 46,XX,del(11)(q23). None of 14 type B G6PD lymphoblastoid lines analyzed in detail contained these karyotypic abnormalities, which strongly suggests that a visible chromosomal alteration is not the sole step in the development of this disease. We hypothesize that at least two events are involved in the pathogenesis of this patient's myelodysplasia: one causing proliferation of a clone of genetically unstable pluripotent stem cells and another inducing chromosomal abnormalities in its descendants.
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PMID:Evidence for a multistep pathogenesis of a myelodysplastic syndrome. 632 94

Lymphocytes from eight preleukemia patients were exposed to Epstein-Barr virus (EBV) in vitro in an attempt to establish lymphoblastoid cell lines. No signs of viral infection were detected, and no cell lines were obtained. Studies using fluorescein-labeled EBV and flow cytometry revealed an unusual and consistent deficiency in EBV receptors in all patients examined. In control studies, about 15% of the unseparated lymphocytes from healthy donors bound fluorescein-labeled EBV. In spite of the lack of EBV receptors, B-lymphocytes amounted to 10 to 20% of the preleukemia lymphocyte populations, a proportion similar to that in healthy donors. When lymphocytes from preleukemic patients were first implanted with functional EBV receptors and then exposed to EBV, synthesis of EBV-determined nuclear, early, and viral capsid antigens was induced. Subsequently, several cell lines originating from preleukemic patients' lymphocytes were established. These lines are of a B-lymphocyte origin and carry EBV genome. They will provide experimental material for the molecular analysis of lymphocytic defects in preleukemia and their possible role in the transition to acute leukemia.
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PMID:Deficiency in Epstein-Barr virus receptors on B-lymphocytes of preleukemia patients. 660 54

After previous serological screening for Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6) and human cytomegalovirus (HCMV) showed elevated antibody titers against EBV and HHV-6 in more than 50% of patients with myelodysplasia and chronic myeloproliferative diseases, the present study was carried out in order to investigate viral antigen expression and distribution in bone marrow cells of these patients. Trephine biopsies were studied from 60 patients with myelodysplasia (MDS), 36 patients with chronic myelogenous leukemia (CML) and 18 patients with osteomyelofibrosis (PMF). Elevated anti-EBV EA titers were found in 62% of the MDS cases, in 33% of the CMLs and in 62% of the OMF patients. HHV-6 titers were elevated in 18% of the MDS cases, but in only one case each of CML and OMF. Antigen expression in bone marrow cells was even more frequent: EBV-EA was 76% in MDS cases, 77% in CML and 40% in OMF. HHV-6 p41 was observed in 47% of the MDS cases, in 54% of the CML cases and in 20% of the OMFs. In comparing these data with those from the literature and with our own studies in Hodgkin's disease, it is hypothesized that the reactivated herpesviruses may contribute to the pathogenesis of these hematopoietic disorders by interfering with the cytokine regulation of cell proliferation and differentiation.
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PMID:Demonstration of active and latent Epstein-Barr virus and human herpevirus-6 infections in bone marrow cells of patients with myelodysplasia and chronic myeloproliferative diseases. 789 80

The clinicopathologic features of 23 patients with hematophagic histiocytosis (HH) are described. All of them exhibited increased histiocytes associated with hemophagocytosis in the marrow. The patients usually presented with fever, hepatosplenomegaly, lymphadenopathy, and cytopenia. The underlying illnesses were heterogeneous, including non-Hodgkin's lymphoma in 17, systemic lupus erythematosus in one, diabetes mellitus in one, acute myelomonocytic leukemia in one, myelodysplastic syndrome in one, and unknown cause in two. Among 17 non-Hodgkin's lymphoma, 14 were peripheral T-cell lymphoma, two were B-cell lymphoma, and one was an undefined phenotype. Among 14 patients with peripheral T-cell lymphoma, six of the patients had nasal T-cell lymphoma. Five of these 14 patients initially diagnosed as malignant histiocytosis turned out to be T-lineage lymphoma after immunophenotypic studies. Active infections, most of viral origin, were documented in eight patients, including Epstein-Barr virus in three, cytomegalovirus in three, herpes simplex virus in three, Pseudomonas aeruginosa in one, Bacteroides vulgatus in one, and mycoplasma in one. Some of them had mixed virus and bacteria infection. Sixteen (70%) of our patients died of their acute illness within 10 weeks of the diagnosis of HH. In the past, the clinical and histologic differentiation between hematophagic histiocytosis and true histiocytic neoplasm (histiocytic medullary reticulosis/malignant histiocytosis) has proved difficult, but now these can be distinguished with immunohistologic, immunogenetic, and cytogenetic studies, especially in the cases of peripheral T-cell lymphoma with hemophagocytic syndrome.
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PMID:Hematophagic histiocytosis: a clinicopathologic analysis of 23 cases with special reference to the association with peripheral T-cell lymphoma. 792 83

A cell line designated SKM-1 was newly established from leukaemic cells of a 76-year-old Japanese male patient with monoblastic leukaemia following myelodysplastic syndrome (MDS). The cells were obtained from peripheral blood of the patient when he lost multiple point mutations of ras genes with acquisition of chromosomal abnormalities during disease progression in MDS. The cells grew as a single floating cell, and have been continuously growing with the morphological characteristics of immature monoblasts by serial passages during the past 42 months with a doubling time of about 48 h. By cytochemical analysis, the cloned cells were positive for butyrate esterase, but negative for the Epstein-Barr virus associated nuclear antigen. Phenotypic analysis revealed the expression of myelomonocyte specific antigens such as CD4, CD13, CD33 and HLA-DR. Cells from the primary peripheral blood and those from 50 passages of the SKM-1 cell line both possessed no activated ras genes but showed karyotype abnormalities with 46,XY, del(9)(q13;q22), der(17) t(17;?)(p13;?). The SKM-1 cells have two mutations in p53 gene and overexpress the p53 products. This cell line may contribute to a better understanding of molecular mechanisms in the progression from MDS to myelogenous leukaemia.
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PMID:Establishment of a leukaemic cell line from a patient with acquisition of chromosomal abnormalities during disease progression in myelodysplastic syndrome. 813 67


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