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)

Erythromelalgia is caused by platelet-mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low-dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia. Here we extend observations on the occurrence of erythromelalgia in thrombocythemia associated with primary myelofibrosis, Philadelphia-chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.
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PMID:Erythromelalgia in thrombocythemia of various myeloproliferative disorders. 834 46

Relapsing polychondritis is a rare disorder of uncertain origin characterized by recurrent inflammation of cartilage. A case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis is reported. A 60-year-old man who had been diagnosed as MDS was admitted because of pain and swelling in the bilateral preauricular regions and cheek. A diagnosis of relapsing polychondritis was made by coexistence of auricular chondritis, arthropathy, ocular inflammation and audio-vestibular disturbance. He also developed ocular palsies and optic neuritis. He was treated with prednisolone, azathioprine, dapsone, and then with steroid pulse therapy. Moreover, plasmapheresis and high dose gamma-globulin therapy were undertaken. However, all these treatments were unsuccessful and he died of respiratory failure.
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PMID:[Relapsing polychondritis in a patient with myelodysplastic syndrome]. 170 34

The endstage in a patient suffering from acute myeloid leukemia secondary to myelodysplastic syndrome was characterized by bleedings, anemia, pain in the upper abdomen and ascites. At autopsy the radicles of the portal veins were occluded by leukemic infiltrates and fibrosis. These lesions block the perfusion of the liver presinusoidally and, together with the anemia (hypoalbuminemia), result in ascites resistant to all therapy.
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PMID:[Stenosis of the terminal portal vein branch in acute myeloid leukemia]. 192 60

1,2-Dimethyl-3-hydroxypyrid-4-one (L1) has been given daily for 1-15 months to 13 transfusion dependent iron loaded patients. No significant change occurred in liver, renal or cardiac function, ECG and radionucleotide angiocardiogram, in audiometry tests and in visual function and electrical retinography. No skin rashes, gastrointestinal symptoms and no neurological changes that could be detected clinically were observed. Two of the patients died of their underlying diseases. One patient had severe cardiac abnormalities before receiving L1 and died of congestive heart failure with infections 5 weeks after stopping a 2-month course of L1. The other, a patient with myelodysplasia suffered recurring infections due to progression of the disease. Joint and muscle pains occurred in five patients. In two these disappeared despite continuing the drug; another patient developed swollen ankle joints which gradually resolved on stopping L1 therapy; a patient with underlying osteoarthritis complained of mild pain and stiffness in her knees which remained intermittent both on and off the drug while in the fifth patient peripheral small joint swelling and pain present before starting L1 improved with L1 therapy. One patient, with Blackfan Diamond anaemia, developed a Lw red cell antibody 6 months after commencing L1. This disappeared on stopping the drug and did not reappear. She then developed severe agranulocytosis and thrombocytopenia 6 weeks after recommencing L1 after 3 months discontinuation of the drug. No other patient showed a change in granulocyte or platelet count.
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PMID:Long-term trial with the oral iron chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1). II. Clinical observations. 209 34

Interferons are new and effective agents in the treatment of various haematological neoplasias. Alpha-interferon (natural or recombinant) has a high efficacy (90% response rate) in hairy cell leukaemia. Complete remissions are, however, rare and definite cure of the disease is unlikely. Alpha-interferon induces haematological remissions in about two thirds of patients with chronic myeloid leukaemia and leads to a reduction in Philadelphia chromosome in about 40% of patients. It is uncertain, however, whether this treatment will actually prolong the life of these patients as compared with conventional treatment. Alpha-interferon has a beneficial effect in some patients with low malignant non-Hodgkin lymphomas (in particular follicular lymphomas). The response rate in myeloma is rather small (20%). Gamma-interferon is not effective in hairy cell leukaemia, non-Hodgkin lymphoma and myeloma. It is, however, of some efficacy in chronic myeloid leukaemia (the response rate in lower than with alpha-interferon) and possibly has some effect in patients with acute myeloid leukaemia and myelodysplastic syndromes. The toxicity of interferons (alpha and gamma) consists of an influenza-like syndrome during the first days of treatment. Low doses of alpha-interferon show virtually no long-term toxicity. However, bone and muscular pain is sometimes dose-limiting with intermediate doses (5 to 15 million units) of alpha-interferon.
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PMID:[Interferon therapy in hematologic neoplasms]. 245 54

One hundred patients 10 years of age or older with myelodysplasia were evaluated to compare located versus dislocated hips with regard to neurologic level, ambulation, hip pain, skin condition, and spinal deformity. Operated and nonoperated individuals were compared. Analysis was carried out to determine what the overall long-time function was in located, dislocated, and "relocated" individuals. Of the 100 patients, 72 presented at follow up with bilateral located hips, 18 with unilateral hip dislocations, and 10 with bilateral dislocations. Twenty patients functioned at T12 levels or above, 30 patients had preservation of anterior thigh musculature, and 25 patients had posterior leg or hip abductor power. Thirteen patients (15 hips) were found to have some degree of pain, 1 patient with bilateral dislocation, 5 with unilateral dislocation, and 7 with bilaterally located hips. Ambulatory function was not affected in any neurologic group by location versus dislocation of the hips. Skin ulceration problems were not increased in patients with hip dislocation. Major spinal deformity in most groups correlated highly with neurologic levels, but not with location versus dislocation of the hips. In one group, an increase in lumbar lordosis was present in unilateral hip dislocations. This study suggests that adolescents and young adults with myelodysplasia have different functional levels related to the neurologic level which are not related to whether the hips are located or dislocated.
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PMID:The role of hip location and dislocation in the functional status of the myelodysplastic patient. A review of 100 patients. 265 80

The availability of rhGM-CSF has allowed the in vivo treatment of patients with cytopenia. Therefore, a phase I-II trial was initiated to study the effect of rhGM-CSF in patients with myelodysplastic syndromes who were not eligible for other kinds of therapy. rhGM-CSF has been tested in 10 patients in doses from 15 micrograms/m2 to 150 micrograms/m2 given intravenously over 8 hours for a cycle of 7 days followed by an interval of 14 days and a second 7-day treatment course. A dose-dependent increase in leukocyte count was observed in 9 of 10 patients. No change in reticulocyte numbers was seen and only one patient experienced an increase in platelet count. Toxicity mainly consisted of mild phlebitis at the site of infusion and sternal pain after bolus injection. An increase in blast cell counts in some patients necessitated the start of low-dose Ara-C therapy.
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PMID:In vitro and in vivo action of recombinant human GM-CSF (rhGM-CSF) in patients with myelodysplastic syndromes. 306 85

The availability of rh GM-CSF has allowed the in vivo treatment of patients with cytopenia. Therefore a phase I/II trial was initiated to study the effect of rh GM-CSF in patients with myelodysplastic syndromes who were not eligible for other kinds of therapy. rh GM-CSF has been tested in 10 patients in doses from 15 micrograms/m2 to 150 micrograms/m2 given intravenously over 8 hours for a cycle of 7 days followed by an interval of 14 days and a second 7-day treatment course. A dose dependent increase in leukocyte count was observed in 9 out of 10 patients. No change in reticulocyte numbers was seen and only one patient experienced an increase in platelet count. Toxicity mainly consisted of mild local phlebitis at the site of infusion and sternal pain after bolus injection. An increase in blast cell counts in some patients necessitated the start of low dose Ara-C therapy.
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PMID:Phase I/II study with GM-CSF in patients with myelodysplastic syndromes. 307 28

Chiari malformations without myelodysplasia are rarely diagnosed in the pediatric age group. With current neurodiagnostic techniques, however, they are being seen more frequently. Unfortunately, the prognosis is not clear because publications have included a number of different entities, used a variety of surgical approaches, and lacked long term follow-up. Sixteen patients younger than 20 years were treated for Chiari malformations (without myelodysplasia) between 1975 and 1985. The average age was 11 years, and the average duration of symptoms was 20 months. The common symptoms were isolated motor weakness (56%), pain (37.5%), and sensory loss (25%). Frequently seen signs were motor deficit (81%), sensory loss (50%), scoliosis (50%), and cranial nerve palsy (50%). The surgical procedures used were foramen magnum decompression (3 transoral clivus odontoid resections and 15 posterior fossa decompressions with dural grafting), alteration of cerebrospinal fluid (CSF) pathways at the cervicomedullary junction (plugging the foramen cecum and a 4th ventricle to subarachnoid shunt with posterior fossa decompression), and ventriculoperitoneal shunting (2 cases). In follow-up, 37.5% of the patients are asymptomatic, 50% are improved, and 12.5% are stable after an average follow-up period of 43 months. The asymptomatic group was younger (9.3 years) and had a shorter symptom duration (7.2 months) than both the improved (11.9 years, 16.4 months) and the stable groups (15 years, 20 months). Optimal outcome depends on complete evaluation of the abnormal CSF pathways and bony abnormalities at the craniovertebral junction. Operation is then directed toward correction of these abnormalities as delineated radiographically. Of our patients, 87.5% have at least shown improvement, which has been long term in all cases.
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PMID:Presentation and management of pediatric Chiari malformations without myelodysplasia. 275 73

A phase-I/II trial was initiated to study the effect of rhGM-CSF in patients with myelodysplastic syndromes who were not eligible for other kinds of therapy, rhGM-CSF was given to 9 patients in doses of 15 micrograms/m2-150 micrograms/m2 as an intravenous 8-h infusion for a cycle of 7 days followed by an interval of 14 days and a second 7-day treatment course. A dose-dependent increase in the leukocyte count was observed in 7 out of 9 patients. No change in reticulocyte numbers was seen and only 1 patient experienced an increase in platelet count. Toxicity mainly consisted of mild local phlebitis at the site of infusion and sternal pain after bolus injection.
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PMID:Recombinant human granulocyte-macrophage colony-stimulating factor in patients with myelodysplastic syndromes--a phase-I/II trial. 328 27


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