1/8. Spontaneous remission of anemia associated with a myelodysplastic syndrome with disease evolution into a myeloproliferative state.A red cell transfusion-dependent patient with a myelodysplastic syndrome had progression into a myeloproliferative state with thrombocytosis. At the same time, the patient became transfusion independent, and a subsequent bone marrow examination revealed a previously undetected loss of chromosome 7. The patient remains well with control of thrombocytosis by anagrelide therapy.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
2/8. Behcet's disease associated with myelodysplastic syndrome: a case report.A rare case of Behcet's disease associated with myelodysplastic syndrome (MDS) is described. A 50-year-old Korean female suffering recurrent oral ulcer, genital ulcer, fatigue, arthralgia in both knees and fever was diagnosed as Behcet's disease. The findings of bone marrow aspirates were consistent with refractory anemia, a subtype of myelodysplastic syndrome. Chromosomal analysis of bone marrow cells revealed 46,XX,-8,-20, der(8)t(8;20)(p23;p10), der(8) t(8;20)(p23;q10)[30]. The chromosomal changes found in this patient were different from those of previous reports, which mostly revealed trisomy 8. If anemia, low reticulocyte count and dyspoietic cells are sustained in Behcet's disease, physicians should be alert to the possibility of MDS with aberration in chromosome 8 and perform a bone marrow study for the proper diagnosis and treatment of the disease. We presented a case of Behcet's disease associated with MDS, which is the first Korean case.- - - - - - - - - - ranking = 1.2keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
3/8. amifostine plus erythropoietin in a patient with low-risk myelodysplastic syndrome.Increasingly, the therapeutic use of hematopoietic growth factors and immunomodulatory agents is under investigation in patients with low-risk myelodysplastic syndrome (MDS). Studies on amifostine therapy-alone or in combination with erythropoietin (EPO)-indicate that long-term treatment is possibly a decisive factor for therapy success. Therefore, we treated an 81-year-old female, transfusion-dependent patient with MDS and refractory anemia (RA) with amifostine and EPO over a period of 2 years. Following a 4-week induction phase of 5 x 500 mg amifostine plus 3 x 10000 IU EPO per week and maintenance therapy of 1 x 500 mg amifostine plus 3 x 10000 IU EPO per week, normal hemoglobin values were reached in week 14. A long-lasting erythroid response could be observed with a reduction of EPO to 2 x 10000 IU and 1 x 10000 IU and, at present, once a week application of amifostine alone (1 x 500 mg). Apart from the 1st week, the treatment was carried out at the outpatient department and was well tolerated by the patient. The patient experienced a good general clinical condition without further need for hospitalization or blood transfusions.- - - - - - - - - - ranking = 1keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
4/8. erythropoietin-resistant anaemia in a predialysis patient with klinefelter syndrome.A diabetic predialysis patient who had significantly reduced sensitivity to erythropoietin therapy was admitted to Tsukuba University Hospital. Many factors that might have been the cause of the erythropoietin resistance were examined, and a diagnosis of refractory anaemia was made based on a bone marrow aspiration biopsy. A cytogenetic abnormality (47, XXY) was also detected in the bone marrow biopsy specimen, and hence the patient was also diagnosed with klinefelter syndrome. It was suspected that the sex chromosome abnormality influenced glucose intolerance, renal insufficiency, and erythropoietin resistance due to myelodysplastic changes in the bone marrow.- - - - - - - - - - ranking = 0.062913114491355keywords = myelodysplastic (Clic here for more details about this article) |
5/8. Disappearance of cytogenetic abnormalities and clinical remission during therapy with 13-cis-retinoic acid in a patient with myelodysplastic syndrome: inhibition of growth of the patient's malignant monocytoid clone.Median survival is as little as 6 months for patients with refractory anemia with excess blasts who demonstrate an abnormal karyotype in the majority of marrow cells. We treated a patient who presented with 29% marrow blasts and 90% abnormal metaphases with 13-cis-retinoic acid. He achieved a complete clinical and cytogenetic remission during therapy. To determine the mechanism of the response, serial studies were done of the effects of 13-cis-retinoic acid and dexamethasone on in vitro growth of his marrow cells. During clinical remission, when the drug was not administered, marrow growth remained significantly depressed. During relapse, the remission growth pattern was replaced by overgrowth of the karyotypically abnormal monocytoid clone. Clonal growth occurred in cultures containing colony-stimulating activity or dexamethasone but was absent in cultures containing concentrations of 13-cis-retinoic acid achieved in vivo. After the drug was reinstituted, a second clinical stabilization developed. Since 13-cis-retinoic acid inhibits normal monocyte colony growth, we postulate that the patient's unusual clinical responses to the drug were due to in vivo growth inhibition of the malignant monocytoid clone.- - - - - - - - - - ranking = 0.8keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
6/8. Bone marrow curettage in myelodysplastic disorders. A stimulus for regeneration in disturbed hematopoiesis.Bilateral bone marrow curettage of the iliac crest was performed in eight patients with marked anemia resulting from various myelodysplastic syndromes. The procedure was performed with the idea that an element of faulty hematopoietic microenvironment existed and that regeneration of normal marrow might occur in areas distant from the site of curettage. Four of the eight failed to respond to this procedure and no long-term response was obtained. One of the "failed" patients had a delayed response 18 months after the curettage was performed. In four patients, the procedure proved to be successful and adequate hemoglobin levels were achieved (10 to 14 g/dL) with reduction of the blood transfusion requirement. The diseases of two of these patients are still in remission 26 to 96 months after the procedure was performed. The procedure is recommended for patients with anemia refractory to conventional therapy and in whom a faulty hematopoietic microenvironment may be present.- - - - - - - - - - ranking = 0.45165245796542keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
7/8. Hematological abnormalities and cholestatic liver disease in two patients with mevalonate kinase deficiency.We describe two patients with mevalonate kinase deficiency and prominent hematologic abnormalities and cholestatic liver disease. Patient R.B. was not anemic at birth, but developed petechiae and cutaneous extramedullary hematopoiesis, hepatosplenomegaly, leukocytosis, and recurrent febrile events without positive bacterial or viral cultures. Patient N.M. manifested minor anomalies, hepatosplenomegaly, anemia, thrombocytopenia, recurrent febrile crises, and facial rashes. Mevalonic aciduria was found by urinary organic acid analysis, and mevalonate kinase deficiency was documented in both. The clinical spectrum of normocytic hypoplastic anemia, leukocytosis, thrombocytopenia, and abnormal blood cell forms led to diagnoses of congenital infection, myelodysplastic syndromes, or chronic leukemia in these patients before recognition of mevalonate kinase deficiency. mevalonate kinase deficiency represents a single-gene abnormality that may be associated with significant hematologic findings. Recognition of the variability of this disorder with some patients manifesting only mild neurologic findings, yet significant hepatosplenomegaly, normocytic anemia, thrombocytopenia, and leukocytosis is important for all specialists who need to be aware of this organic aciduria.- - - - - - - - - - ranking = 0.2keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |
8/8. pseudomonas aeruginosa orbital phlegmon in a patient treated for myelodysplastic syndrome with concomitant sjogren's syndrome.pseudomonas aeruginosa orbital infections have been described very rarely in patients with neutropenia after chemotherapy. We report the case of a woman with the unusual association of Sjogren's disease and myelodysplasia, who suffered from a pseudomonas aeruginosa orbital phlegmon after chemotherapy for her myelodysplastic syndrome. Partial intestinal antibiotic decontamination with ciprofloxacine did not prevent the infection. She was treated successfully with intravenous ceftazidime, netilmicin and granulocyte-colony stimulating factor (G-CSF). The normalization of the granulocyte count seems to play a crucial role for recovery. We present the clinical and radiological findings, discuss the therapy and review the literature concerning ocular infections due to Pseudomonas. Other infections due to this germ in immunocompromised hosts are briefly reviewed.- - - - - - - - - - ranking = 0.45165245796542keywords = myelodysplastic syndrome, myelodysplastic (Clic here for more details about this article) |