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 56-year-old African-American man presented with fever of unknown origin and peripheral blood and bone marrow findings of myelodysplastic syndrome (MDS): refractory anemia with an excess of blasts in transformation that subsequently progressed to acute myeloblastic leukemia (AML). Ultrastructural study of two bone marrow specimens having the findings of MDS revealed frequent, large tubuloreticular structures (TRS) in lymphocytes, plasma cells, macrophages, and endothelial cells. Several cylindrical confronting cisternae (CCC) were present in macrophages and an endothelial cell. Two partially developed CCC were present in a plasma cell. TRS and CCC were not observed in eight subsequent bone marrow specimens obtained during the 9-month course of the AML. This is the first reported occurrence of TRS and CCC in MDS. These inclusions are probably related to an unidentified viral infection or possibly to cytokines released by the dysplastic hematopoietic cells.
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PMID:Cytomembranous inclusions in myelodysplastic syndrome. 133 38

The effectiveness of sulbactam/cefoperazone (SBT/CPZ) on severe infections associated with hematological diseases was evaluated in a nation-wide multicenter clinical study. SBT/CPZ (4-6 g/day), a 1:1 combination of SBT and CPZ, was given intravenously to 437 patients with hematological disorders. The underlying diseases included acute nonlymphocytic leukemia, acute lymphocytic leukemia, malignant lymphoma, multiple myeloma, myelodysplastic syndrome and others. Thus, 94.3% of the patients had hematological malignancies. The complicating infections included sepsis in 41 cases; sepsis suspected in 205; pneumonia in 47; urinary tract infection in 15; fever of unknown origin in 59; and others in 70. Clinical efficacies of SBT/CPZ were as follows; markedly effective, 83 cases; effective, 170; fairly effective, 59; and ineffective, 110. The efficacy rate (markedly effective plus effective) was 60.0% as a whole. The efficacy rate of SBT/CPZ in sepsis and suspected cases, which accounted for 56.3% of the infections, was 59%. Mild side effects such as skin rash were observed in 15 patients (3.1%). As for abnormal laboratory test results, transient increases in GOT, GPT, A1-P, LDH, etc. were observed in 42 patients (8.6%). Therefore, SBT/CPZ is considered to be a useful drug in empiric therapy for severe infections associated with hematological diseases.
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PMID:[Clinical evaluation of sulbactam/cefoperazone for severe infections associated with hematological disorders]. 196 Aug 59

The authors studied 35 marrow biopsies from 32 patients with rheumatoid arthritis, systemic lupus erythematosus, mixed connective tissue disease, polymyositis, and psoriatic arthritis. Reasons for biopsy included cytopenia, fever of unknown origin, and malignancy. Cellularity was abnormal in 71%. Plasma cells were increased in 60% and associated with lymphoid aggregates. Immunoperoxidase stains showed polyclonal perivascular plasma cells and increased T-cells forming lymphoid aggregates. Two patients had granulomas without documented infection. Anemic patients had findings consistent with anemia of chronic disease, erythroid aplasia, hemolysis, and iron deficiency. Iron stores were variable. Platelet and granulocyte precursors were variably altered and did not predictably correlate with the presence, absence, or cause of thrombocytopenia and neutropenia. Myelodysplastic syndromes were present in two patients with rheumatoid arthritis. Osteomalacia and osteoporosis were seen, resulting from renal failure and steroids. Marrow findings are unpredictable and reflect the diverse causes of cytopenias in patients with connective tissue disorders.
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PMID:Bone marrow findings in connective tissue disease. 281 17

The frequencies, kinds, pathogens, and risk factors of infections in the myelodysplastic syndromes (MDS) were analysed in 430 cases. The overall tendency was for one infectious episode per 1023.5 patient days. The frequency of infectious episodes was highest just after diagnosis of MDS when more than 4 episodes per 1000 patient days occurred. Thereafter, the rate declined rapidly to about 0.3 episodes per 1000 patient days within 4 years. The most frequent infection was that of the respiratory tract followed by sepsis and fever of unknown origin (FUO). Among the types of infection resulting in death, sepsis and FUO comprised the highest proportion (40%) followed by respiratory tract infections (39%). The most frequent pathogen observed was Staphylococcus bacteria. The significant multivariate risk factors for fatal infections were subtype, hemoglobin, dependence on red blood cell transfusion, age, and sex. A staging system was created using these five simple variables at diagnosis.
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PMID:Infection in myelodysplastic syndromes before evolution into acute non-lymphoblastic leukemia. 794 62

A bone marrow investigation is a common examination in HIV infected patients for the study of cytopenia, febrile syndromes of unknown origin and extension of neoplastic disorders. A study was made of bone marrow specimens from 35 patients with advanced HIV infection (stage IC or C, CDC, Atlanta) for morphologic and culture investigations (aerobes, anaerobes, fungi, and mycobacteria). In nine patients cytopenia accounted for the investigation of bone marrow specimens (9 aspirates and 3 biopsies); in only two cases did the investigation orientate towards a possible etiology: in the first patient a parvovirus B19 infection and in the second patient a hemophagocytic syndrome. In twenty-five patients the bone marrow specimen was studied because of fever of unknown origin (23 aspirates and 10 biopsies) and only in one case was the identification of Mycobacterium tuberculosis obtained. The other patient was studied for lymphoma staging and aspirate and biopsy examinations were normal. A high percentage of patients had eosinophilia, plasmacytosis, increased iron reserves, fibrosis, and changes consistent with myelodysplasia. In conclusion, in our experience the investigation of bone marrow specimen was of little help to clarify the possible etiology of cytopenia and febrile syndromes of unknown origin in patients with advanced HIV infection.
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PMID:[Usefulness of bone marrow examination in patients with advanced HIV infection]. 870 Oct 58

A case of acute myeloid leukaemia presenting as pyrexia of unknown origin and weight loss with pancytopenia is described. Initial investigations revealed trilineage myelodysplasia which evolved into acute myeloid leukaemia within 2 weeks of presentation. He was commenced on a standard induction regimen consisting of idarubicin, Ara-C and thioguanine. Throughout his hospital stay he remained febrile. In spite of exhaustive investigations no cause for the pyrexia was found nor did he respond to any form of treatment. He died after 9 weeks in hospital. His post-mortem examination revealed widespread disseminated tuberculosis without any reactive inflammatory tissue response or granuloma formation.
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PMID:Acute myeloid leukaemia complicated by anergic tuberculosis. 911 7

Topotecan is a topoisomerase I inhibitor with significant activity in patients with myelodysplastic syndrome and chronic myelomonocytic leukemia. Pre-clinical data suggest a synergistic activity with DNA damaging agents such as cyclophosphamide, where topotecan might prevent the repair of cyclophosphamide-induced DNA damage. We thus designed a combination including cyclophosphamide 500 mg/m2 every 12 hours given on days 1 to 3; topotecan 1.25 mg/m2/day by continuous infusion on days 2 to 6, and cytosine arabinoside (ara-C) 2 g/m2 over 4 hours daily for 5 days on days 2 to 6 (CAT). Sixty six (63 evaluable) patients were treated. Fifty two patients had refractory (n=12) or relapsed (n=40) acute myelogenous leukemia (AML), and eleven had acute lymphocytic leukemia (ALL) (refractory n=3, relapsed n=8); their median age was 57 years (range, 18 to 79 years). Eleven patients (17%) achieved a complete remission (CR), and two patients (3%) had a hematologic improvement (HI; met all criteria for CR except for platelets < 100x10(9)/L), for an overall response rate of 20%. Responses occurred in 12 of 52 AML patients (23%), including 10 CR (19%) and 2 HI (4%), and in 1 of 11 patients with ALL (9%). Myelosuppression was universal; there were 23 episodes of pneumonia or sepsis and 18 episodes of fever of unknown origin complicating 74 courses of CAT. Non-hematologic toxicity was mostly gastrointestinal, including nausea, vomiting, diarrhea and mucositis, but was severe in only 8%. In summary, the CAT regimen is well tolerated and has significant anti-leukemia activity which warrants further investigation.
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PMID:Cyclophosphamide, ara-C and topotecan (CAT) for patients with refractory or relapsed acute leukemia. 1078 92

Primary MDS is a group of heterogenous clonal haematopoetic disorders. In a third of patients MDS terminates as acute myeloid leukaemia, usually resisitant to treatment, while the others succumb due to infections and haemorrhage. Conservative managements of MDS (chemotherapy, haematopoetic growth factors, modulation of cytokine network) are unsuccessful, while the bone marrow transplantation is the only definite treatment. We reviewed clinical and haematological presentations, frequency of dysplastic features, histological and cytogenetic findings in 29 children with primary MDS. Indications for haematological evaluation in our patients were symptoms and signs of isolated or combined cytopenias, fever of unknown origin and frequent infections. Hepatosplenomegaly was found in 19 (65%) patients, while this pattern was found in 10% of adult patients. Normochromic anaemia was found in 25 (86%) patients and thrombocytopenia in 23 (76%). Patients presenting pancytopenia had the lowest probability of survival. Degree of dysplasia, histology and kariotype of bone marrow had no influence on survival rates. Prognostic factors in paediatric MDS are of limited significance, as MDS in children is an absolute indication for bone marrow transplantation.
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PMID:[Primary myelodysplastic syndrome in children]. 1563 84

Fever of unknown origin (FUO) is a common clinical diagnostic dilemma. In the elderly, causes of FUO most commonly include malignancy or infection, and less commonly include collagen vascular diseases. Among the collagen vascular diseases causing FUO in the elderly, polymyalgia rheumatica/temporal arteritis, and adult Still's disease (adult juvenile rheumatoid arthritis) are difficult diagnoses to prove. Among the infectious causes of FUO in the elderly are subacute bacterial endocarditis, intra-abdominal abscesses, and extrapulmonary tuberculosis. In the elderly, neoplastic causes of FUO include lymphomas, hepatomas, renal cell carcinomas, and hepatic or central nervous system metastases. Acute leukemias, particularly during "blast" transformation, may present as acute fevers in the absence of infection, but are rare causes of FUO. Preleukemia/myelodysplastic syndromes are exceedingly rare causes of FUO. We present a case of an elderly man who presented with findings that initially suggested adult Still's disease. Prolonged and profound monocytosis provided the key clue to his subsequent diagnosis of preleukemia/myelodysplastic syndrome. In this patient, a positive Naprosyn test result also suggested a neoplastic cause for his FUO. After months of prolonged fevers, myelocytes/metamyelocytes were eventually demonstrated in his peripheral smear during hospital evaluation. These findings, in concert with the persistent monocytosis, highly elevated ferritin levels, polyclonal gammopathy on serum protein electrophoresis, and eventual presence of myelocytes/metamyelocytes on peripheral smear, prompted a bone marrow test that demonstrated blast cells confirming the diagnosis of preleukemia myelodysplastic syndrome as the cause of this patient's FUO.
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PMID:Fever of unknown origin due to preleukemia/myelodysplastic syndrome: the diagnostic importance of monocytosis with elevated serum ferritin levels. 1686

The intravascular variant of diffuse large B-cell lymphoma (IVBL) is a rare form of non-Hodgkin lymphoma that is frequently diagnosed at autopsy because the symptoms are nonspecific or confusing. We report a case of IVBL in a woman with pre-existing myelodysplastic syndrome manifested as a fever of unknown origin, bilateral adrenal enlargement and subsequent development of panhypopituitarism. Lymphomatous infiltration or osteomyelitis of the sella was supposed. Despite antibiotic and corticosteroid therapy the patient died within three months. An intravascular variant of B-cell lymphoma with intravascular collections of lymphomatous cells predominantly localized in the adrenal and pituitary glands was found at autopsy. The association of panhypopituitarism with bilateral adrenal enlargement is uncommon in endocrinological praxis and the occurrence of combined endocrine involvement in a patient with IVBL has not been described in previous literature.
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PMID:Intravascular variant of diffuse large B-cell lymphoma with combined endocrine involvement. 1686 48


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