Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective analysis we assessed the data of 46 patients with myelodysplastic syndromes (MDS), who had received more than 50 blood transfusions during the course of disease. The number of units given ranged from 50 to 155 (mean 79). 20 patients (RA n = 4, RARS n = 12, RAEB n = 1, RAEB/T n = 2, CMML n = 1), followed up between 8 and 108 months (mean survival time 39.4 months), developed a secondary hemochromatosis. More than 40% of the patients showed signs of heart failure, in some cases accompanied by cardiac arrhythmias. 11 patients also suffered from hepatopathy and 5 developed diabetes mellitus. Secondary hemochromatosis was particularly common in patients with RARS. Refractory congestive heart failure secondary to hemochromatosis was the cause of death in 14 patients, whereas none died from hepatic insufficiency. We conclude that the risk of secondary hemochromatosis should not be neglected in polytransfused patients with MDS. In some cases, particularly those with favorable prognostic features of MDS, it may shorten life expectancy. The availability of a new oral iron chelator (1,2-dimethyl-3-hydroxypyrid-4-one or L1) offers a promising and practicable approach to prevent this complication.
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PMID:[Secondary hemochromatosis in polytransfused patients with myelodysplastic syndromes]. 128 62

123 patients with small cell lung cancer (SCLC) presented to the National Cancer Center Hospital (Tokyo) between 1978 and 1986. 22 of 71 patients with limited stage disease (LD) and none of 52 patients with extensive disease (ED) survived for 3 years. 15 of the 22 three year survivors had significant late complications. All patients received chemotherapy and either thoracic irradiation, resection or both. No prophylactic cranial irradiation was given. 4 patients developed cardiac failure, 2 with a dilated cardiomyopathy, despite the fact that no patient received over 420 mg/m2 of doxorubicin. 12 patients of the 17 who received thoracic irradiation developed radiation pneumonitis and 3 required hospitalisation for severe haemoptysis (2) or cavity formation (1). 1 patient who received nimustine developed a fatal myelodysplastic syndrome and 2 additional patients developed second primary tumours in the oesophagus (1) and stomach (1). Mild peripheral neuropathy (WHO grade 1) was persistent in 3 patients and asymptomatic azotemia (WHO grade 1) in 7. Despite advances in the treatment of SCLC there are very few asymptomatic long-term survivors.
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PMID:Late toxicities and complications in three-year survivors of small cell lung cancer. 185 18

Of 511 cases of brucellosis studied between December 1983 and February 1986, four (0.8%) had sternoclavicular (STCL) arthritis. Two were male and two female, and only one was younger than 50 years old. All four cases had significantly high specific IgG antibody titres (1 of 1280), measured by the indirect immunofluorescent (IIF) test, and two had Brucella melitensis isolated from their blood. In two cases, STCL arthritis was the presenting problem, and it was associated in one with ankle arthritis, hepatitis, renal impairment, orogenital ulcers and a haematological picture of myelodysplasia; in the other it was a relapsing STCL arthritis. In the remaining two cases, STCL arthritis was part of an extensive osteoarticular disease, which was associated in one with cachexia, liver cirrhosis, heart failure and prostatitis with urine retention, and in the other with severe thrombocytopenia. Excellent results were obtained from six to eight weeks' therapy with streptomycin, rifampicin and cotrimoxazole or tetracycline.
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PMID:Brucellar sternoclavicular arthritis, the forgotten complication. 325 Mar 41

Treatment of myelodysplastic syndromes (MDS) with recombinant human erythropoietin (Epo) is successful in only 10% to 25% of patients. We performed a pilot study in 10 anaemic patients with MDS to examine whether sequentially applied recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF) and Epo improves haemoglobin levels and/or reduces red blood cell transfusion requirements. Morphological diagnoses of patients were refractory anaemia (RA) in 3 cases, RA with ring sideroblasts in 3 cases and RA with excess blasts in 4 cases. GM-CSF was given subcutaneously at a dose of 150 micrograms/m2/d during the initial 10 days. From day 11, Epo was administered by subcutaneous injections for 8 weeks at a dose of 100 U/kg/d and subsequently at an escalated dose of 200 U/kg/d in 3 patients. Changes in reticulocyte counts, haemoglobin levels, RBC support and ferrokinetic parameters were compared with pretreatment values. Two out of 8 evaluable patients showed a rise in haemoglobin levels at week 8 and 10, respectively, and lost their transfusion dependency for a period of 13 and 27 weeks. In 1 patient, haemoglobin level increased only after dose escalation of Epo (200 U/kg/d). Leukocyte counts remained uneffected by treatment with Epo, while 1 patient showed a 4-fold increase in platelet numbers. Toxicity was mild. Two patients died of pneumonia and global heart failure, respectively, unrelated to growth factor therapy. Based on this pilot study, we conclude that sequential treatment with GM-CSF and Epo does not increase erythroid responses in anaemic patients with MDS. Because of the delayed increase in haemoglobin in both responders, we surmise that the beneficial effects were induced by Epo alone.
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PMID:Sequential administration of recombinant human granulocyte-macrophage colony-stimulating factor and human erythropoietin for treatment of myelodysplastic syndromes. 785 74

We examined the efficacy of thalidomide in 34 patients with myelodysplastic syndromes (MDS): five RAEB-T, four RAEB, three CMML, six RARS, and 16 RA. Patients belonged to the following cytogenetic groups: 15 complex abnormal karyotypes, 12 normal karyotypes, four cases with 5q- as sole anomaly and three single aberrations. The median thalidomide dose was 400 mg/day (25/34 patients). Four patients discontinued the study after less than 5 weeks, because of fatigue (three) or skin rash (one). One patient died of heart failure after 4 weeks. In the remaining 29 patients (median follow-up: 13 months), treatment responses were classified according to the IWG criteria. Six patients (four RA, two CMML) showed progressive disease (five with transformation into AML) and four patients showed stable disease. Hematological improvement (HI) was observed in 19 patients. Nine of the responders (three RA, one RARS, two RAEB, three RAEB-T) achieved partial remission with granulocytes > or = 1500/microl, Hb > 11 g/dl and platelets > or =100,000/microl. Four patients (one RARS, one CMML, one RAEB, one RAEB-T) had a major response, with platelet and RBC transfusion independence. Six patients (five RA, one RARS) showed minor responses (three HI-E, two HI-E+HI-P, one HI-E+HI-N). Hematological improvement occurred after a median of 2 months of thalidomide treatment. Two patients (RAEB-T) relapsed after a partial remission lasting 8 and 16 months, respectively. In summary, a therapeutic benefit was achieved in 19 of 34 study patients (56%).
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PMID:Thalidomide for the treatment of patients with myelodysplastic syndromes. 1184 Feb 56

We analyzed data of 76 consecutive patients with myelodysplastic syndrome (MDS) and isolated del(5q) (n=66) or del(5q) plus one additional chromosomal abnormality (n=10) included in our MDS database over the last 26 years. The median age of our patient population was 66.8 years. The male to female ratio was 1:1.7. In all, 14 patients (18%) had advanced MDS with an increased medullary blast count. A total of 17 patients (22%) had significant dysplasia in the nonmegakaryocytic cell lines. Nearly half of the study population showed erythroid hypoplasia in the bone marrow. The projected median survival of patients with isolated del(5q) is 146 months for a median follow-up of 67 months. Patients with an increased medullary blast count and those with an additional chromosomal abnormality have a significantly shorter overall survival (24 and 45 months, respectively) than patients with isolated del(5q). We did not find survival differences for different cytogenetic breakpoints, nor did the amount of dysplasia have an impact on survival in our population. In total, 29 patients have died. Deaths occurred primarily due to transformation into acute leukemia, infection, or cardiac failure. Our data support the current definition of a separate entity of MDS with del(5q) that has been suggested by the World Health Organization.
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PMID:Clinical, morphological, cytogenetic, and prognostic features of patients with myelodysplastic syndromes and del(5q) including band q31. 1458 79

This review outlines the main chelator groups studied to date, and the evidence for their clinical effectiveness. For each treatment, the strength of evidence was documented according to the guidelines from the American College of Cardiology and the American Heart Association. Three main haematological diseases were considered as models: thalassaemia major, sickle-cell disorders and myelodysplasia. Although the data in the literature do not allow firmly evidence-based conclusions, the findings suggest that in thalassaemia major: (i) deferoxamine remains the drug of choice for chelation treatment; (ii) if there is deferoxamine intolerance or a change of treatment is suggested, the options are deferiprone or, if the liver iron concentration is high, deferasirox treatment; and (iii) if the ferritin level is >2500 microg/l and liver iron concentation is >7 mg/g/dry weight, continuous subcutaneous (s.c.) or intravenous (i.v.) deferoxamine, or combined treatment with deferiprone and deferoxamine is advised. In case of heart failure, there is currently more solid documentation to support continuous s.c. or i.v. deferoxamine treatment than combined treatment with deferiprone and deferoxamine. However, more recent data in the literature suggest that the latter could be a satisfactory alternative. Finally, if iron chelation is required for sickle-cell disorders or myelodysplastic syndromes, the current data support the use of deferoxamine treatment.
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PMID:Light and shadows in the iron chelation treatment of haematological diseases. 1765 52

We performed MRI and endocrine assessment in 27 adult Chinese patients with severe aplastic anemia (SAA) and myelodysplasia (MDS). Despite high ferritin levels, cardiac hemosiderosis and heart failure was not common, and were not concordant in most cases. However, significant correlation was found between cardiac T2/T2* MRI assessment, ejection fraction and ferritin levels. Furthermore, there was a high incidence subclinical pancreatic abnormality that was related to liver MRI T2* readings. Hence, chelating agents may still have a role to improve organ function. Prospective data with serial functional and imaging monitoring is needed.
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PMID:A pilot MRI study of organ specific hemosiderosis and functional correlation in Chinese patients with myelodysplasia and aplastic anemia with raised ferritin levels. 1848 75

(1) Imatinib, a tyrosine kinase inhibitor, was first marketed for the treatment of chronic myeloid leukaemia and some gastrointestinal stromal tumours. Its indications have gradually expanded over the years. (2) There is no consensus treatment for adults with Philadelphia chromosome-positive acute lymphoblastic leukaemia. In a trial comparing imatinib versus chemotherapy as initial treatment for 55 patients, the haematological response rate was higher with imatinib. Non-comparative trials provided haematological response rates of about 90%, but it is not known whether or not this translates into a survival advantage. In three non-comparative trials including patients with relapsed or refractory disease after chemotherapy, 50% of patients showed a survival time of at least 7 months. In the absence of any direct comparisons, we do not know if this represents an improvement over the results obtained with the best palliative care. (3) The only potential cure for myelodysplastic syndromes is allogeneic haematopoietic stem cell transplantation but this is not always feasible. Some myelodysplastic syndromes are associated with myeloproliferation and PDGFR gene rearrangements; imatinib is the first drug available for these patients. The manufacturer has compiled data on 55 patients treated with imatinib. Most patients had a favourable haematological response, but no survival data were published. (4) The severity of the hypereosinophilic syndrome is highly variable. Forms associated with FIP1L1-PDGFRalpha gene rearrangements have a poor prognosis. Clinical evaluation of imatinib in this setting is based on a compilation of data concerning 176 treated patients. The haematological response rate was high in patients with the mutant gene, but it is not known whether this translated into increased survival. (5) Dermatofibrosarcoma protuberans is a rare form of mainly localised skin cancer. Treatment is based on surgical excision but relapses are frequent. In one series, 9 out of 12 patients treated with imatinib had at least a partial tumour response, making surgical excision possible in 3 cases. (6) Imatinib has diverse and frequent adverse effects; nausea and vomiting, oedema, fluid retention, cutaneous disorders and heart failure. There is some evidence pointing to a risk of urologic cancers and altered bone metabolism.
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PMID:Imatinib: new indication. New indications, but not robust evidence. 1862 99

Many patients with myelodysplastic syndromes (MDS) have severe anaemia. However, regular blood transfusions, which are widely used to maintain quality of life and prevent anaemia-related morbidity and mortality, have a negative impact on survival as a result of iron overload. Retrospective surveys have shown an association of transfusion dependence with hepatic, pituitary, and pancreatic dysfunction, cardiac failure, and cardiac death. Survival is significantly decreased in transfusion-dependent patients, and the main cause of non-leukaemic death is cardiac failure. However, iron chelation therapy reduces serum ferritin levels and is associated with significantly improved survival in patients with MDS. Current guidelines recommend starting iron chelation therapy after 25-50 units of blood have been transfused, or when serum ferritin levels rise above 1,000-2,000 microg/L. The patients who are most likely to benefit from iron chelation therapy are those who have low-risk disease (International Prognostic Scoring System low or intermediate-1 risk) with a life expectancy of more than 1 year. More specific studies in patients with MDS are needed to evaluate the impact of iron chelation therapy on morbidity and mortality, and provide a stronger evidence base for treatment guidelines.
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PMID:The deleterious effects of iron overload in patients with myelodysplastic syndromes. 1905 54


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