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)

A 72-year-old man was admitted for examination of dyspnea and pitting edema of the lower legs in July, 1996. His hemoglobin level was 6.9 g/dl, and myelodysplastic syndrome (MDS) was revealed by bone marrow aspiration, and frequent transfusions were needed. His renal function rapidly deteriorated in the middle of August (BUN 45 mg/dl, Cr 4.8 mg/dl) and IgA nephropathy (IgAN) with marked intestinal nephritis was disclosed by renal biopsy. In November, joint manifestations of warmth and pain, which suggested arthritis, appeared at the bilateral wrist and ankle joints. Soon after receiving prednisolone (20 mg/day), the arthritis was relieved. Renal function also improved (BUN 41 mg/dl, Cr 21 mg/dl) and frequent transfusions were no longer necessary. This is a case with various clinical manifestations of MDS, IgAN, and arthritis, and appears to be the first MDS case complicated with IgAN. A number of case reports have identified immune abnormalities in patients with MDS. Immune and bone marrow abnormalities have been reported to be involved in the pathogenesis of IgAN. Thus, MDS could be complicated by IgAN. Their pathogenetic association is discussed in this paper.
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PMID:[A case of myelodysplastic syndrome associated with IgA nephropathy]. 1046 57

We aimed to perform a prospective analysis of the main characteristics of deaths occurring in the oncohaematology department of a general hospital. From November 1995 to February 1997, a total of 81 patients died in our unit, 50 of whom (61.7%) were male. Their mean age was 67.8 (range 19-96) years. Underlying diseases were: multiple myeloma (9 cases), acute myeloid leukaemia (22), lymphoma (14), chronic lymphocytic leukaemia (6), acute lymphoblastic leukaemia (4), myelodysplastic syndromes (3), solid tumours (11), and other (12). The previous disease duration ranged from 5 days to 276 months (mean 31.9 months). The duration of the last hospital stay varied between 0 (death on arrival or on way to hospital) and 40 days (mean 9.3 days). Two patients died in the emergency unit just before entering our department (1 suicide). Only 15 patients had been admitted for the first time. In 70% of these cases death appeared predictable, as the consequence of refractory or end-stage disease. In these cases, all the "do not resuscitate" orders were in place at least 48 h before death. About half the patients died without any relative present. The frequencies of the clinical complaints evaluated were the following: pain necessitating opiates 27%; infection- or disease-related fever 40%; dyspnoea 44%; haemorrhage 20%; CNS disturbances 25%. The percentages of use of therapy tools chosen as indicators were: benzodiazepines 80%; chemotherapy 46%; anti-infectious agents 47%; transfusions 42%; major analgesics 27%; and steroids 40%. The circumstances and quality of patient deaths must be regularly evaluated so that palliative care in the final stages of life can be improved.
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PMID:Characteristics of deaths in a department of oncohaematology within a general hospital. A study of 81 cases. 1092 70

A 43-year-old man with no past history presented with symptoms of fever, cough and dyspnoea arising from invasive pulmonary aspergillosis and was found to have myelodysplastic syndrome with monosomy 7. Before initiation of chemotherapy, he deteriorated rapidly, developing multi-organ failure requiring mechanical ventilation, and he eventually succumbed despite amphotericin B treatment. The importance of monosomy 7 in determining immune function in patients with myelodysplastic syndrome is emphasised.
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PMID:Myelodysplastic syndrome with monosomy 7 and pulmonary aspergillosis. 1110 46

A case of 68 years old women suffering from chronic anemia, myelodysplastic syndrome and treated with progestogen due to endometrial hypertrophy is presented. Initially she was admitted to a regional hospital because of progressive weakness and exertional dyspnea. Three months earlier she reported an episode of acute dyspnea and chest pain. On the basis of clinical symptoms and perfusion lung scintigraphy pulmonary embolism (PE) was diagnosed. Patient received i.v. heparin which was changed to s.c. nadroparine subcutaneously. Platelet count dropped to 55,000'/ml on fifth day of treatment from initial level of about 200,000'/ml. Heparin induced thrombocytopenia was diagnosed, heparin was stopped and ticlopidine was recommended. After 3 weeks symptoms suggesting recurrent PE were observed. The patient was transferred to National Tuberculosis and Lung Diseases Research Institute. Recombinant hirudine (Refludan) was administrated (bolus 0.4 mg/kg and initial dose of infusion 0.1 mg/kg/h) overlapping with acenocoumarol from second day. Dose of r-hirudine was adjusted to achieve APTT prolongation 1.5 to 2.5 times of mid-normal range. During treatment with r-hirudine no bleeding and new thromboembolic complications occurred. Platelets count remained within normal range. After 14 days clinical improvement was observed, though symptoms of right ventricular overload and hypoxemia were still present after 6 months of treatment with oral anticoagulants suggesting chronic thromboembolic pulmonary hypertension.
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PMID:[Recombinant hirudine in suspected heparin induced thrombocytopenia--case report of pulmonary embolism]. 1143 91

A 72-year-old man who had worked as a coal worker for 28 years and as a tunnel construction worker for 18 years was admitted because of fever, dyspnea, and appetite loss. Pneumoconiosis had been diagnosed when he was 64 years old and myelodysplastic syndrome at 71 years of age. After admission, the patient was treated with antibiotics and anti-fungal drugs but did not respond. Respiratory failure gradually worsened and he died. Autopsy specimens revealed that the cause of death was exacerbation of respiratory failure due to pulmonary proteinosis rather than pulmonary infection. This is a case of pulmonary proteinosis complicated with pneumoconiosis and myelodysplastic syndrome which, to our knowledge, is rare. We also considered the possibility that defective pulmonary macrophage function due to myelodysplastic syndrome and long-term silica inhalation played a part in the development of pulmonary alveolar proteinosis in this case.
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PMID:[A case of pulmonary alveolar proteinosis complicated with pneumoconiosis and myelodysplastic syndrome]. 1172 94

In a 64-year-old man suffering from hypoblastic myelodysplastic syndrome a secondary acute myeloid leukaemia developed. After induction chemotherapy with resulting partial remission he received an allogenic (related) peripheral blood stem cell transplantation conditioned with 2 Gy total body irradiation. After haematopoietic reconstitution chest pain and dyspnoea appeared. Computer tomography revealed diffuse bilateral infiltrates which were considered to be suspicious for an invasive pulmonary aspergillosis of the left upper lobe. Respiratory and circulatory insufficiency occurred. In bronchoalveolar lavage fluid Aspergillus antigen was detected. In addition, Aspergillus flavus was isolated on Sabouraud-dextrose agar. Ambisome (liposomal encapsulated amphotericin B) was applied in high dosages. On the skin of the sides and the back five livid red stained nodular lesions with haemorrhagic infarctions appeared. Pathohistologically, both in PAS (periodate acid Schiff) and Grocott-Gomori staining conglomerates of septated hyphae were detected in corium and subcutis. In addition, Aspergillus flavus grew from skin tissue. Despite antifungal treatment the patient died from Aspergillus pneumonia and generalized aspergillosis with dissemination to heart, brain, and skin.
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PMID:Secondary cutaneous aspergillosis due to Aspergillus flavus in an acute myeloid leukaemia patient following stem cell transplantation. 1260 97

A 71-year-old Japanese male with myelodysplastic syndrome progressing to overt leukaemia and hepatocellular carcinoma developed dyspnea and urticaria immediately after infusion of platelet concentrate (PC). He exhibited an identical reaction following blood transfusion. Serum haptoglobin was undetectable. The patient was determined to be homozygous for Hp(del) by polymerase chain reaction (PCR). Antibody to haptoglobin was detected by enzyme-linked immunosorbent assay (ELISA) and Western blot analysis. No antibodies against human leucocyte antigen (HLA) or platelet-specific antigens were detected. Washed PC and washed red blood cells were effective in preventing the transfusion-related anaphylactoid reactions.
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PMID:Effectiveness of washed platelet concentrate and red cell transfusions for a patient with anhaptoglobinemia with antihaptoglobin antibody. 1196 40

A retrospective survey was conducted over a 10-year period (1990-99) among 52 haematology divisions in order to evaluate the clinical and laboratory characteristics and outcome of patients with proven Pneumocystis carinii pneumonia (PCP) complicating haematological diseases. The study included 55 patients (18 with non-Hodgkin's lymphoma, 10 with acute lymphoblastic leukaemia, eight with acute myeloid leukaemia, five with chronic myeloid leukaemia, four with chronic lymphocytic leukaemia, four with multiple myeloma, three with myelodysplastic syndrome, two with myelofibrosis and one with thalassemia) who developed PCP. Among these, 18 (33%) underwent stem cell transplantation; only two received an oral prophylaxis with trimethroprim/sulphamethoxazole. Twelve patients (22%) developed PCP despite protective isolation in a laminar airflow room. The most frequent symptoms were: fever (86%), dyspnoea (78%), non-productive cough (71%), thoracic pain (14%) and chills (5%); a severe hypoxaemia was present in 39 patients (71%). Chest radiography or computerized tomography showed interstitial infiltrates in 34 patients (62%), alveolar infiltrates in 12 patients (22%), and alveolar-interstitial infiltrates in nine patients (16%). Bronchoalveolar lavage was diagnostic in 47/48 patients, induced sputum in 9/18 patients and lung biopsy in 3/8 patients. The diagnosis was made in two patients at autopsy. All patients except one started a specific treatment (52 patients trimethroprim/sulphamethoxazole, one pentamidine and one dapsone). Sixteen patients (29%) died of PCP within 30 d of diagnosis. Multivariate analysis showed that prolonged steroid treatment (P < 0.006) and a radiological picture of diffuse lung involvement (P < 0.003) were negative diagnostic factors.
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PMID:Pneumocystis carinii pneumonia in patients with malignant haematological diseases: 10 years' experience of infection in GIMEMA centres. 1197 21

We have published previously a prototype of a decision model for anaemic patients with myelodysplastic syndromes (MDS), in which transfusion need and serum erythropoietin (S-Epo) were used to define three groups with different probabilities of erythroid response to treatment with granulocyte colony-stimulating factor (G-CSF) + Epo. S-Epo </= 500 U/l and a transfusion need of < 2 units/month predicted a high probability of response to treatment, S-Epo > 500 U/l and >/= 2 units/month for a poor response, whereas the presence of only one negative prognostic marker predicted an intermediate response. A total of 53 patients from a prospective study were included in our evaluation sample. Patients with good or intermediate probability of response were treated with G-CSF + Epo. The overall response rate was 42% with 28.3% achieving a complete and 13.2% a partial response to treatment. The response rates were 61% and 14% in the good and intermediate predictive groups respectively. The model retained a significant predictive value in the evaluation sample (P < 0.001). Median duration of response was 23 months. Scores for global health and quality of life (QOL) were significantly lower in MDS patients than in a reference population, and fatigue and dyspnoea was significantly more prominent. Global QOL improved in patients responding to treatment (P = 0.01). The validated decision model defined a subgroup of patients with a response rate of 61% (95% confidence interval 48-74%) to treatment with G-CSF + Epo. The majority of these patients have shown complete and durable responses.
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PMID:A validated decision model for treating the anaemia of myelodysplastic syndromes with erythropoietin + granulocyte colony-stimulating factor: significant effects on quality of life. 1264 74

Increased bone marrow angiogenesis and vascular endothelial growth factor (VEGF) levels are adverse prognostic features in patients with acute myeloid leukemia (AML) or myelodysplastic syndromes (MDSs). VEGF is a soluble circulating angiogenic molecule that stimulates signaling via receptor tyrosine kinases (RTKs), including VEGF receptor 2 (VEGFR-2). AML blasts may express VEGFR-2, c-kit, and FLT3. SU5416 is a small molecule RTK inhibitor (RTKI) of VEGFR-2, c-kit, and both wild-type and mutant FLT3. A multicenter phase 2 study of SU5416 was conducted in patients with refractory AML or MDS. For a median of 9 weeks (range, 1-55 weeks), 55 patients (33 AML: 10 [30%] primary refractory, 23 [70%] relapsed; 22 MDS: 15 [68%] relapsed) received 145 mg/m2 SU5416 twice weekly intravenously. Grade 3 or 4 drug-related toxicities included headaches (14%), infusion-related reactions (11%), dyspnea (14%), fatigue (7%), thrombotic episodes (7%), bone pain (5%), and gastrointestinal disturbance (4%). There were 11 patients (20%) who did not complete 4 weeks of therapy (10 progressive disease, 1 adverse event); 3 patients (5%) who achieved partial responses; and 1 (2%) who achieved hematologic improvement. Single agent SU5416 had biologic and modest clinical activity in refractory AML/MDS. Overall median survival was 12 weeks in AML patients (range, 4-41 weeks) and not reached in MDS patients. Most observed toxicities were attributable to drug formulation (polyoxyl 35 castor oil or hyperosmolarity of the SU5416 preparation). Studies of other RTKI and/or other antiangiogenic approaches, with correlative studies to examine biologic effects, may be warranted in patients with AML/MDS.
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PMID:SU5416, a small molecule tyrosine kinase receptor inhibitor, has biologic activity in patients with refractory acute myeloid leukemia or myelodysplastic syndromes. 1264 63


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