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)

Patients with the following diagnoses were presented: pyoderma gangraenosum in a patient with myelodysplastic syndrome passing into an acute myelomonocytic leukemia and specific cutaneous infiltration, primary genital infection with herpes simplex virus, type 1 (HSV-1) in an adult patient, pellagroid, Sweet's syndrome with follicular involvement, Sweet's syndrome in a patient with cancer of the breast, lichen amyloidosus, angiolymphoid hyperplasia with eosinophilia, Darier's disease 1. associated with basal cell carcinoma 2. with specific cutaneous infiltrations in a patient with acute myeloid leukemia, body building, anabolic steroids and fertility, multiple trichodiscomas and perifollicular fibromas, Buschke's scleroedema adultorum, extensive necrobiosis lipoidica without diabetes mellitus, extramammary, multifocal type of Paget's disease.
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PMID:[52d Cologne Dermatology Meeting of the Cologne University Dermatology Clinic 24 January 1990]. 198 79

The use of supralethal chemoradiotherapy followed by marrow transplantation has progressed from being an experimental approach applied only to a limited number of end-stage patients to an important therapeutic option appropriate for many adults with a variety of hematologic malignancies. With the use of transplantation, 10% to 30% of patients with relapsed leukemia and approximately 50% of patients with acute nonlymphoblastic leukemia in first remission can be cured. Cures have also been seen in a variety of other hematologic malignancies, including chronic granulocytic leukemia, preleukemia, hairy cell leukemia, and malignant lymphoma. Transplantation is currently limited by the need for a suitable marrow donor; by the complications of the transplant procedure, including infection, graft-versus-host disease, and the toxicities of intensive chemoradiotherapy; and by the risk of recurrent disease. Some of these limitations will likely be overcome as a result of current research. The use of partially matched family members and matched unrelated donors will make transplantation available to more patients. Some forms of posttransplant infection, including those associated with herpes simplex and cytomegalovirus, can now be prevented or treated. Improved methods of controlling graft-versus-host disease including T-cell depletion of marrow and the use of more effective immunosuppressive agents, as well as a better understanding of the toxicities of the preparative regimens, are making the transplant procedure safer and more tolerable. Finally, the development of better preparative regimens and transplantation earlier in the patient's disease course will likely allow for a larger percentage of patients to be cured.
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PMID:Treatment of acute leukemia in adults with chemoradiotherapy and bone marrow transplantation. 388 38

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 72-year-old male suffering with MDS was admitted to our hospital because of slowly progressive dementia, convulsions and consciousness disturbance. A CT scan of the brain showed a low density area in the right temporal to parietal lobe and T2-weighted MRI of the brain revealed a high intensity signal in the same area. Herpes simplex virus DNA was detected in CSF by PCR method. He was diagnosed as having herpes simplex encephalitis but his clinical course was prolonged and considered atypical as herpetic infection in CNS. However, after administration of aciclovir, progression of his symptoms stopped, and a CT scan still revealed abnormal findings with the same area after more than four months. We thought his chronic course of herpes encephalitis was caused by incomplete immune function suppressed by MDS.
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PMID:[A case of chronic herpes encephalitis with myelodysplastic syndrome (MDS)]. 820 Jan 42

To control severe GvHD while maintaining strong GvL effects in the context of allo-stem cell transplantation (allo-SCT), a phase I/II clinical trial of infusions of donor lymphocytes transduced with the herpes simplex virus thymidine kinase (TK-DLI) started at the Tsukuba University Hospital. To date, five (2 AML, 2 ALL, and 1 MDS) out of eight patients enrolled in the trial received approximately 7x10(7) transduced cells per kilogram of body weight and four patients showed some clinical responses such inhibition of the leukemic cell proliferation or mitigation of lymph node swelling. Especially, one MDS patient achieved complete remission and has remained in CR for 2 years after the treatment. GvHD developed in two patients (1 acute and 1 chronic) and the acute (grade III) was successfully controlled by administration of ganciclovir without any immunosuppressive drugs. Since HSV-TK as a strong antigen induced CTLs against transduced cells in patients, however, TK-DLI is expected to provide a more effective adoptive immune cell therapy by performance just after allo-SCT where the patient's immune function is severely damaged.
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PMID:Gene and cell therapy for relapsed leukemia after allo-stem cell transplantation. 1850 42

More and more transcription factors and their motifs have been reported and linked to specific gene expression levels. However, focusing only on transcription is not sufficient for mechanism research. Most genes, especially in eukaryotes, are alternatively spliced to different isoforms. Some of these isoforms increase the biodiversity of proteins. From this viewpoint, transcription and splicing are two of important mechanisms to modulate expression levels of isoforms. To integrate these two kinds of regulation, we built a linear regression model to select a subset of transcription factors and splicing factors for each co-expressed isoforms using least-angle regression approach. Then, we applied this method to investigate the mechanism of myelodysplastic syndromes (MDS), a precursor lesion of acute myeloid leukemia. Results suggested that expression levels of most isoforms were regulated by a set of selected regulatory factors. Some of the detected factors, such as EGR1 and STAT family, are highly correlated with progression of MDS. We discovered that the splicing factor SRSF11 experienced alternative splicing switch, and in turn induced different amino acid sequences between MDS and controls. This splicing switch causes two different splicing mechanisms. Polymerase Chain Reaction experiments also confirmed that one of its isoforms was over-expressed in MDS. We analyzed the regulatory networks constructed from the co-expressed isoforms and their regulatory factors in MDS. Many of these networks were enriched in the herpes simplex infection pathway which involves many splicing factors, and pathways in cancers and acute or chronic myeloid leukemia.
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PMID:Discovering transcription and splicing networks in myelodysplastic syndromes. 2424 32

We present the case of a patient, with history of myelodysplastic syndrome and recent bone marrow transplant, who developed fulminant liver failure secondary to herpes simplex virus (HSV) hepatitis. His presentation was unique, as findings of liver microabscesses on computed tomography scan have not been described previously in this patient population. Despite initial treatment with acyclovir, he continued to deteriorate, and later sensitivities found the HSV strain to be resistant to acyclovir. HSV hepatitis with secondary liver failure is rare and, without appropriate treatment, its mortality is >80%. Early suspicion and immediate therapy are the keys to improve patient survival.
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PMID:A unique presentation of acute liver failure from herpes simplex virus hepatitis. 2722 30

Infections with adenovirus (AdV) and herpesviruses can result in considerable morbidity and mortality in pediatric hematopoietic stem cell transplant (SCT) recipients. Herpes simplex virus (HSV) reactivations are usually prevented by acyclovir (ACV) prophylaxis, whereas cidofovir (CDV) has been used off indication to manage AdV infections. We report a child with myelodysplastic syndrome undergoing multiple SCT, who experienced HSV-1 disease including severe mucositis and herpetic whitlow, as well as high viral load AdV DNAemia. Both ACV and CDV were ineffective; however, viral loads were decreased with brincidofovir, resulting in viral clearance. A subsequent Epstein-Barr virus disease with relevant meningoencephalitis responded to rituximab.
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PMID:Brincidofovir clearance of acyclovir-resistant herpes simplex virus-1 and adenovirus infection after stem cell transplantation. 2748 52