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)

Sweet's syndrome is known often to associate with non-lymphocytic leukemia (ANLL); however, there have been very few reports of Sweet's syndrome associated with myelodysplastic syndrome (MDS). It was reported that improvement and exacerbation of these two syndromes occurred simultaneously. We present here a 49-year-old male with Sweet's syndrome developed in RAEB in T. He complained of fever and infiltrative eruptions on the trunk and legs. At the time of admission to Tsukuba University Hospital, the peripheral blood showed leukocytopenia (WBC 2,000/microliter: Blast 9%, PMN 51%) and anemia (Hb 6.5 g/dl). Pseudo-Pelger anomaly of neutrophils was found on the blood smear. From the hematological findings and the result of skin biopsy, the patient was diagnosed as having MDS (RAEB in T) complicated by Sweet's syndrome. Prednisolone was effective to improve his fever and eruptions. However, when treated with low-dose Ara-C and when transformed into acute myelogenous leukemia, there was no correlation between the condition of Sweet's syndrome and the percentages of blasts in the marrow. We suggest that eruptions of Sweet's syndrome associated with MDS are not always a good index of exacerbation of MDS.
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PMID:[Appearance of Sweet's syndrome in a patient with myelodysplastic syndrome (MDS) without relation to the hematological findings of MDS]. 279 96

A 5-year-old girl was diagnosed as having idiopathic thrombocytopenic purpura (ITP) based on symptoms of nasal bleeding and purpura. The platelet count was 35,000/microliters without anemia or leukopenia. Micromegakaryocytes were observed in normocellular bone marrow without dyserythropoiesis or dysgranulopoiesis. She had periosteal fibroma of the rib and atopic dermatitis with elevated serum IgE. Prednisolone and azathioprine were administered but with no response. The cumulative dose of azathioprine was 20 g for 28 months. Nine years after the diagnosis of ITP, she was admitted because of dyspnea and anemia. The white cell count was 26,900/microliters with 17% monocytes. The hemoglobin was 3.9 g/dl and the platelet count was 9,000/microliters. Dyserythropoiesis, dysgranulopoiesis and micromegakaryocytes were observed in hypercellular bone marrow. The chromosome analysis demonstrated 47, XX, +21. She was diagnosed as having chronic myelomonocytic leukemia (CMMoL) and received bone marrow transplantation (BMT) from an HLA-identical sibling conditioned with high-dose busulfan and melphalan. After 17 months of remission, the disease recurred with an abnormal karyotype of 47, XX, +21, 7q+. Despite a second BMT conditioned with high-dose etoposide, cyclophosphamide and total body irradiation, she died of the disease. Refractory thrombocytopenia as a subgroup of myelodysplastic syndrome, rather than ITP, might have preceded the development of CMMoL, with the possibility of azathioprine-induced leukemia.
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PMID:[Chronic myelomonocytic leukemia developed 9 years after the diagnosis of idiopathic thrombocytopenic purpura in a child]. 807 97

An 80-year old man was diagnosed as having immune thrombocytopenic purpura based on epistaxis, purpura and by the platelet count 8 x 10(9)/l. Prednisolone and gamma globulin were administered and the platelet count had been kept around 50 x 10(9)/l during his follow up. Two years from the onset of immune thrombocytopenic purpura he was admitted because of leukocytosis (79 x 10(9)/l with 79% monocytes), anemia and thrombocytopenia. Hypercellular bone marrow with dysplasia of three lineages was observed. In the bone marrow cytogenic analysis, a -6, clonal cytogenic abnormality was observed. 45XY, der(6), t(6;6)(q16;q23). He was diagnosed as having chronic myelomonocytic leukemia. This is a difficult case in which it was diagnosed as refractory thrombocytopenia as a subgroup of myelodysplastic syndrome, rather than immune thrombocytopenic purpura. which might have preceded the development of chronic myelomonocytic leukemia.
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PMID:Chronic myelomonocytic leukemia developed 2 years after the onset of immune thrombocytopenic purpura like syndrome. 1112 16

Autoimmune hemolytic anemia (AIHA) rarely occurs in myelodysplastic syndrome (MDS). A 36-year-old Asian female was diagnosed with MDS (refractory cytopenia with multilineage dysplasia, RCMD) and complicated by AIHA 7 months later. Secondary myelofibrosis developed at the same time. Steroid therapy was ineffective and cyclosporin A (CsA) was discontinued due to its neurotoxicity with the development of leukoencephalopathy. However, the patient achieved a good hematological response after the use of mycophenolate mofetil (MMF, CellCept) with a dose of 1 g/day and prednisolone (15 mg/day). Prednisolone was tapered off over the next 3 weeks. The patient did not require any blood support 4 weeks after the use of MMF and has been hematologically stable for 4 months. To our knowledge, this is the first report of using MMF in treating MDS complicated by AIHA. MMF might be considered as a salvage therapy for patients with refractory anemia complicated by AIHA.
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PMID:Myelodysplastic syndrome complicated by autoimmune hemolytic anemia: remission of refractory anemia following mycophenolate mofetil. 1248 69

We describe the successful treatment of pyoderma gangrenosum (PG) that developed in a patient with myelodysplastic syndrome (MDS). A 63-year-old Japanese man with MDS was admitted to our hospital because of a large skin ulcer on his neck in November 2001. The initial diagnosis was infectious dermatitis, and antimicrobial therapy was performed, using imipenem/cilastatin, isepamicin, and amphotericin B. However, this therapy was not effective, and the lesion worsened. Cultures of blood, throat swab, and ulcer pus yielded no microorganisms. A biopsy of the skin lesion revealed a severe infiltration of neutrophils in the dermis, without any evidence of infection. The lesion was finally diagnosed as PG, and systemic administration of corticosteroid hormone was started in December 2001. The patient was initially pulsed with 1 g methylprednisolone daily for 3 days. The dose was immediately reduced, and the treatment was maintained with 30 mg prednisolone daily. The skin lesion responded markedly to the therapy, and C-reactive protein became negative. The patient was discharged in February 2002 because the lesion was almost cured. Prednisolone administration was tapered after 6-month maintenance therapy. No recurrence of PG was seen, although his MDS transformed into leukemia in April 2003. Only 31 cases of MDS developing PG have been reported in the past 20 years in Japan. This report describes one such rare patient who was successfully treated with the use of high-dose pulse methylprednisolone and long-term maintenance therapy.
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PMID:Successful treatment of pyoderma gangrenosum that developed in a patient with myelodysplastic syndrome. 1451 99

A 31-year-old pregnant woman was referred to our hospital due to anemia and thrombocytopenia, and was diagnosed as having myelodysplastic syndrome (refractory anemia) with autoimmune thrombocytopenia. Administration of high dose methylprednisolone and gamma-globulin did not raise her platelet count, and she subsequently delivered a healthy baby after the transfusion of a large amount of platelets. Although the anemia spontaneously improved after delivery, the platelet count remained unchanged. Prednisolone was thus administered a second time, which did finally increase the platelet count. This is the first reported case of a pregnant woman with myelodysplastic syndrome in whom corticosteroid administration was effective for thrombocytopenia.
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PMID:[Successful corticosteroid treatment of thrombocytopenia in a pregnant woman with myelodysplastic syndrome (refractory anemia)]. 1519 47

A 57-year-old man underwent an autologous hematopoietic stem cell transplant for mantle cell lymphoma in August 1999. Anemia and thrombocytopenia appeared in November 2001. He was diagnosed with further hematological examination as having acute myeloid leukemia with multilineage dysplasia following secondary myelodysplastic syndrome. He received the allogeneic hematopoietic stem cell transplant from his HLA DRB1 locus mismatched brother in May 2002. The nonmyeloablative preparative regimen consisted of fludarabine 30mg/m2 for 6 days and busulfan 4mg/kg for 2 days. Eosinophilia, decrease of lacrimal fluid and liver dysfunction appeared on Day 104. We diagnosed this as chronic GVHD and treated the patient with prednisolone 10 mg/day. Thereafter, his chronic GVHD gradually improved. He had fever and myalgia in the extremities and lumbar region with elevated serum CPK and aldolase in January 2003. Histological examination led to a diagnosis of polymyositis simultaneously with chronic GVHD. Prednisolone 50 mg/day as an initial dose was started for the polymyositis following which the prednisolone dose was gradually tapered off. The polymyositis improved promptly after the administration of prednisolone and remains in remission with a current maintenance program of prednisolone 5 mg/day.
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PMID:[Chronic GVHD with polymyositis after non-myeloablative stem cell transplantation]. 1644 Aug 6

A 64-year-old man had been admitted to a previous hospital because of melena and a diagnosis of ulcerative colitis (UC, pancolitis type) had been made. He had received prednisolone and 5-ASA but steroid-induced hyperamylasemia had developed. Prednisolone had been tapered and halted, but it had resulted in UC relapse and thrombocytopenia. Then, he was referred to our hospital due to severe melena with hypovolemic shock. However, he was also positive for CMV antigen. Thus, we attempted to treat him with ganciclovir for CMV and intravenous cyclosporine (CsA) for UC. According to his clinical course, a reduction of CsA blood concentration induced leukocytopenia. Myelodysplastic syndrome (MDS, RAEB-1) was then revealed after bone marrow biopsy. A high blood CsA concentration may cause the improvement of UC and MDS conditions.
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PMID:A case of ulcerative colitis accompanied with myelodysplastic syndrome successfully treated by cyclosporine. 2181 44