Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The recent recognition of genetic defects in telomeres and telomere repair in multiple human diseases has practical implications for hematologists and oncologists and their patients; consequences for future clinical research in hematology and other subspecialties; and even importance in the interpretation of animal experiments involving cell propagation. Telomere diseases include constitutional marrow failure as dyskeratosis congenita, some apparently acquired aplastic anemia, myelodysplasia and acute myeloid leukemia; pulmonary fibrosis; and hepatic nodular regenerative hyperplasia and cirrhosis. Accelerated telomere attrition is a likely pathophysiology of cancer arising from chronic inflammation. Telomerase can be modulated by sex hormones, which may explain the activity of androgens in marrow failure. Measurement of telomere length of peripheral blood leukocytes is a simple screening clinical assay. Detection of a mutation in a patient has implications for therapy, prognosis, monitoring, and genetic counseling. For research in hematology and oncology, telomere biology could be assessed as a risk for secondary malignancies and in graft-versus-host disease, for progression in a variety of blood cancers, and as potentially modifiable by hormone replacement strategies.
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PMID:Telomere biology and telomere diseases: implications for practice and research. 2123 67

The incidence of hepatocellular carcinoma is increasing. Most patients present beyond potentially curative options and are usually affected by underlying cirrhosis. In this scenario, transarterial therapies, such as radioembolization, are rapidly gaining acceptance as a potential therapy for hepatocellular carcinoma and liver metastases. Radioembolization is a catheter-based liver-directed therapy that involves the injection of micron-sized embolic particles loaded with a radioisotope by use of percutaneous transarterial techniques. Cancer cells are preferentially supplied by arterial blood and normal hepatocytes by portal venous blood; therefore, radioembolization specifically targets tumor cells with a high dose of lethal radiation and spares healthy hepatocytes. The antitumor effect mostly comes from radiation rather than embolization. The most commonly used radioisotope is yttrium-90. The commercially available devices are TheraSphere (glass based; MDS Nordion, Ottawa, Canada) and SIR-Sphere (resin based; Sirtex, Lane Cove, Australia). The procedure is performed on an outpatient basis. The incidence of complications is comparatively less than other locoregional therapies and may include nausea, fatigue, abdominal pain, hepatic dysfunction, biliary injury, fibrosis, radiation pneumonitis, gastrointestinal ulcers, and vascular injury. However, these complications can be avoided by meticulous pretreatment assessment, careful patient selection, and adequate dosimetry. This article focuses on both the technical and clinical aspects of radioembolization with emphasis on patient selection, uses and complications.
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PMID:Radioembolization for primary and metastatic liver cancer. 2193 59

The telomeropathies are a newly described group of human diseases based on the genetics and molecular biology of the telomeres, the ends of chromosomes. Telomeres are repeated hexanucleotides and their associated proteins; the protect chromosomes from recognition as damaged DNA, and their inevitable gradual loss with DNA replication is harmless as they are noncoding. However, when telomeres become critically short in a cell, senescence, apoptosis, or, rarely malignant transformation results. In individuals with mutations in genes involved in telomere repair, especially the enzymatic telomerase complex, telomere attrition is accelerated. Severe deficiencies result in dyskeratosis congenita, a congenital aplastic anemia with associated mucocutaneous abnormalities. Mutations in TERT, the catalytic component, and TERC, the RNA template, can behave as risk factors for the development of bone marrow failure, pulmonary fibrosis, and hepatic cirrhosis. Both penetrance and organ specificity are variable and not well understood. Chromosome instability is a result of critical shortening of telomeres and cancer. For example, short telomeres are the major prognostic risk factor for clonal evolution to myelodysplasia and acute leukemia. Practically, hematologists need to recognize the multisystem presentation of telomere disease, implications for outcomes, and options for therapy.
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PMID:Bone marrow failure and the new telomere diseases: practice and research. 2250 70

Organ transplant recipients are at an increased risk for subsequent malignancies including hematologic malignancies. The development of acute myeloid leukemia (AML) after solid organ transplantation is a rare but well-documented event. It is thought to be a consequence of immune dysregulation secondary to the use of immunosuppressive agents. Herein, we present the management of a liver transplantation recipient who presented with AML and comprehensively review the relevant literature. A 59-year-old male patient presented with fever and cough eight years after an orthotopic liver transplantation for cirrhosis and hepatocellular carcinoma. He received methylprednisolone and mycofenolate mofetil (MMF) followed by tacrolimus and rapamycin as immunosuppression. Upon admission to our hospital, his peripheral blood demonstrated 34% blasts and pancytopenia. A bone marrow biopsy confirmed the diagnosis of myelodysplastic syndrome (MDS) in transformation to AML. He was treated with induction chemotherapy and his sirolimus was continued but he expired four weeks after from refractory disease. No specific guidelines exist for the treatment of AML in solid organ transplant recipients. Treatment should be individualized and concurrent use of chemotherapeutic and immunosuppressive agents should be carefully balanced.
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PMID:Acute myeloid leukemia following solid organ transplantation: case report and comprehensive review. 2266 74

Hepatic regenerative macronodules observed in hepatic cirrhosis are sensitive to ischemia. Lenalidomide is a thalidomide analog used for the treatment of myelodysplastic syndromes, with pleiotropic activities including induction of apoptosis, inhibition of angiogenesis and broad immunomodulatory effects. It has been approved by the Food and Drug Administration (FDA) in the United States and by the European Medicines Agency (EMEA) in 2007 for the use in combination with dexamethasone in the treatment of relapsed or refractory multiple myeloma. We report a unique observation, which strongly suggests the role of Lenalidomide in hepatic regenerative macronodules infarction.
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PMID:Lenalidomide-induced regenerative macronodules infarction in a cirrhosis patient. 2349 74

A 71-year-old man with myelodysplastic syndrome (MDS) receiving treatment with azacitidine developed extensive watery diarrhea for three consecutive days. As a result of high-grade dehydration, the patient was urgently admitted to the hospital and fluid replacement therapy was initiated. However, the patient's diarrhea did not improve. Vibrio cholerae non-O1/non-O139 was detected in a fecal culture. On the fourth day, the patient died due to circulatory collapse. An autopsy revealed extensive necrosis of the intestinal mucosa. Vibrio cholerae non-O1/non-O139-induced diarrheal disease often develops in patients with hepatic cirrhosis and has a serious clinical course. We herein report a fatal outcome of Vibrio cholerae O67 infection in an immunocompromised MDS patient.
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PMID:Fatal diarrheal disease caused by Vibrio cholerae O67 in a patient with myelodysplastic syndrome. 2385

Thrombocytopenia is a common hematologic disorder. Stimulation of thrombopoiesis may reduce the risk for thrombocytopenia-induced bleeding, prevent severe thrombocytopenia, and reduce the need for platelet transfusion. The key cytokine is thrombopoietin (TPO). It regulates proliferation and maturation of megakaryocytes as well as platelet production. TPO is synthesized in the liver. Development of TPO from the laboratory into a therapeutic tool has turned out to be an unexpected challenge. Clinical trials on first-generation thrombopoietic growth factors were stopped in 2001. At present, second-generation thrombopoiesis-stimulating agents have only been approved as orphan drugs for third-line therapy of patients with chronic immune thrombocytopenia. Larger groups in need are patients with myelodysplastic syndrome, chemotherapy-induced thrombocytopenia, other forms of hereditary and acquired bone marrow failure, hepatitis C infections, or liver cirrhosis.
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PMID:Clinical indications for thrombopoietin and thrombopoietin-receptor agonists. 2427 85

Haemochromatosis is due to excessive accumulation of iron in tissues and organs impairing their function. The most common haematologic disorders that are subject to an intensive transfusion regimen bringing excess iron in the body are: thalassemia and myelodysplastic syndrome. The value of serum ferritin in these patients (indicator of iron stores condition) reaches high values. Red cell substitution bringing additional iron intake must be accompanied by administration of chelation therapy in order to prevent haemochromatosis and related complications. We present the case of a patient with thalassemia intermedia, integumentary secondary haemochromatosis, cirrhosis with haemochromatosis, and secondary diabetes, who died at the age of 33 years because of upper gastrointestinal bleeding due to the rupture of oesophageal varices.
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PMID:Secondary haemochromatosis in a patient with thalassemia intermedia. 2479 Dec 10

Endoscopic submucosal dissection (ESD) has been successfully performed in thrombocytopenic conditions such as in patients with liver cirrhosis but successful ESD for early gastric cancer (EGC) in hematologic diseases has rarely been reported. A 52-year-old male patient, who had previously been diagnosed with myelodysplastic syndrome 2 years ago, was admitted to our hospital for ESD of EGC. ESD was performed successfully in this patient after platelet concentrates transfusion on the day of ESD. ESD might be an option for the treatment of EGC in thrombocytopenia due to hematologic diseases when optimal supportive managements are applied.
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PMID:Endoscopic submucosal dissection for early gastric cancer in a patient with myelodysplastic syndrome. 2579 81

We describe two hemodialysis patients with high-risk myelodysplastic syndrome (MDS) treated with azacitidine. A 65-year-old woman (case 1) received azacitidine at 75 mg/m(2) for 7 days, and a 52-year-old man (case 2) with liver cirrhosis received a 70% dose of azacitidine. Both cases developed grade 4 cytopenia, but they achieved transfusion independence after 3 and 2 courses, and the durations of remission were 10 and 11 months, respectively. Case 1 had the complication of febrile neutropenia (FN) twice during the 1(st) and 2(nd) courses, but continued to receive azacitidine treatment thereafter. Case 2 developed infectious peritonitis during the sixth course, and azacitidine treatment was thus discontinued. After a 4-month treatment interruption, he became transfusion-dependent, and re-induction of azacitidine was successful. Of note, the course of case 1 was complicated by erythema nodosum on admission, which then disappeared after one course of azacitidine treatment. The mean durations of hospitalization were 17.5 and 23 days per course of azacitidine treatment, respectively. Though there are few reports of azacitidine treatment for hemodialysis patients with high-risk MDS, we advocate administering azacitidine to such patients, while paying close attention to the dose intensity of azacitidine and taking prompt action to manage infectious complications.
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PMID:Efficacy and adverse events of azacitidine in the treatment of hemodialysis patients with high-risk myelodysplastic syndrome. 2759 16


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