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)

Myelodysplastic changes have recently been described in patients with the acquired immunodeficiency syndrome (AIDS). The authors report a patient with AIDS-related complex and myelodysplasia who rapidly progressed to acute myeloblastic leukemia. Infection with human T-cell lymphotropic virus type III (HTLV-III) was documented by culture and by serology. Chromosome studies showed monosomy of chromosome 7 and structural abnormality of the long arm of chromosome 3. The association of HTLV-III infection with myelodysplasia and acute myeloblastic leukemia may have been coincidental in this reported case, but it is also possible that the leukemia was secondary to the HTLV-III infection. Further investigation appears justified.
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PMID:Myelodysplasia progressing to acute myeloblastic leukemia in an HTLV-III virus-positive homosexual man with AIDS-related complex. 346 25

We have reviewed the causes and risk factors for early death in a group of 295 children who underwent any form of first bone marrow transplantation (BMT) between 1978 and 1992. The commonest indications for transplantation were acute lymphoblastic leukaemia 80 (27.1%), neuroblastoma 69 (23.3%), immune deficiency 57 (19.3%) and myeloid leukaemias/myelodysplasia 50 (16.9%). There were 120 (40.6%) allogeneic BMTs, 118 (40%) autologous BMTs, while 51 (17.2%) children usually with severe combine immune deficiency received BMT from a non-HLA-identical parent, sibling or other relative (FBMT). Two were from identical twins and four from matched unrelated donors (MUD). Thirty-three children (11.2%) died in the first 100 days; the main causes of death being infection (n = 5), relapse (n = 7), graft failure (n = 4), GVHD (n = 7) and organ failure with or without infection (n = 6). There was no significant change in the incidence of early deaths in the three successive 5 year periods (1978-82, 1983-87, 1988-92) although there was some shift in the causes. Infections were the commonest cause during the first 5 year period, relapses followed by GVHD in the second period and single organ failure followed by GVHD and infections in the third period. The main causes of early death were relapse after high-dose chemo/radiotherapy and autologous BMT (7 of 9 deaths) and GVHD and infection after allogeneic BMT (9 of 13 deaths). In the group of 51 children undergoing FBMT there were five deaths from infection, three from graft failure, one from organ failure and one from GVHD.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Early deaths in children undergoing marrow ablative therapy and bone marrow transplantation. 771 76

Infections, an important determining factor in the clinical course of myelodysplastic syndromes (MDS), result in activation of myelomonocytic cells. In this study we demonstrate activation-associated immunophenotypic changes of cell surface antigens on monocytes and granulocytes observed in two groups of MDS patients, one with low and another one with high clinical risk, and compared them to healthy individuals. Significantly changed expression of the complement receptors 1 (CD35) and 3 (CD11b), the Fc gamma receptor I (CD64), the leucocyte-homing receptor (CD44) and the activation associated membrane proteins CD67 and M5 were found on monocytes and/or granulocytes of MDS patients. In low-risk MDS patients we observed activation-associated phenotypic changes only in monocytes, whereas in high-risk MDS patients, both monocytes and granulocytes showed such changes. Additionally, we performed respiratory burst experiments and observed an impaired response of monocytes and granulocytes derived from MDS patients. Despite the fact that all patients were free of infection by clinical criteria, cell surface phenotyping as well as the reduced respiratory burst capacity of myelomonocytic cells suggests in vivo preactivation of these cells.
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PMID:Immunophenotypic characterization of myelomonocytic cells in patients with myelodysplastic syndrome. 810 71

Infection with human immunodeficiency virus type 1 (HIV-1) primarily involves a subgroup of T-lymphocytic cells, but other cell types are also invaded by the virus, including cell lines within the haematopoietic system. Together with infectious, inflammatory and neoplasic processes, invasion of haematopoietic tissue explains the haematological alterations which are seen during the course of infection with HIV-1. Anaemia develops in the large proportion of patients. Thrombocytopenia frequently occurs during the course of the disease, but may be seen in some patients already at the time of diagnosis, where the condition may be misdiagnosed as "idiopathic" thrombocytopenic purpura. Neutropenia is seen in all disease stages, but is most severe in patients with advanced disease. Bone marrow changes include varying degrees of dysplasia in one or more cell lines, which in some patients may mimic a myelodysplastic syndrome. The number of plasma cells is always increased. In many patients the bone marrow stroma exhibits an increased amount of reticular fibres. HIV-1 infection is associated with an increased risk of non-Hodgkin malignant lymphoma. Acute myelogenous leukaemia and myelomatosis have been described in patients with advanced disease. Treatment of the above mentioned haematological abnormalities aims primarily at reducing replication of HIV-1, thereby diminishing suppression of haematopoiesis by the virus infection, and at controlling the opportunistic infections during the course of the disease. Specific antiviral therapy (AZT) is most successful in correcting thrombocytopenia. The possibility of bone marrow suppression mediated by a toxic drug effect should always be considered in this patient group.
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PMID:[Hematological changes associated with human immunodeficiency virus (HIV-1) infection]. 831 70

High serum concentrations of procalcitonin (PCT) have been found during bacterial and parasitic infections. This is a report of two cases of disseminated aspergillosis with moderate PCT increase in two 14-year-old girls after bone marrow transplantation (BMT) for myelodysplastic syndrome and Fanconi's anemia, respectively. In contrast, the important rise of serum CRP observed in these patients tends to demonstrate that the synthesis of these two proteins is under different control mechanisms.
Infection
PMID:Serum procalcitonin rise is only slight in two cases of disseminated aspergillosis. 964 9

The efficacy and safety of topotecan and high-dose cytarabine were evaluated in 59 patients with advanced myelodysplastic syndromes (MDS) (n = 38) or chronic myelomonocytic leukemia (CMML) (n = 21). Topotecan 1.25 mg/m2/d was administered by continuous Intravenous infusion daily for 5 days, and cytarabine 1.0g/m2/d by Infusion over 2 hours for 5 days. At a median follow-up duration of 7 months, 59 patients were evaluable for response and toxicity. Complete remission (CR) was achieved in a significantly larger proportion of MDS patients than CMML patients (66% v 48%, P < or = .05). CR rates for good-risk and poor-risk MDS patients were similar (79% and 58%, respectively). Treatment was particularly effective in patients with poor-prognosis karyotypes and secondary MDS, producing CR rates of 63% and 69%, respectively. Medlan duration of CR was 32 weeks (41 weeks for MDS; 33 weeks for CMML). Median survival was 60 weeks for MDS patients and 41 weeks for CMML patients. Prospective analysis of response by International Prognostic Scoring System (IPSS) risk classification showed comparable CR rates among intermediate 1 (60%), Intermediate 2 (83%), and high-risk (56%) categories. Fever of undetermined origin and Infections occurred in 72% and 59% of patients, respectively. Six patients (10%) died during Induction therapy. This regimen was well tolerated and was associated with a low mortality rate.
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PMID:Results of topotecan-based combination therapy in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia. 1062 23

Forty-five patients with high-risk myeloid malignancies (32 acute myeloid leukemia and 13 high-risk myelodysplastic syndromes) were treated with fludarabine, cytarabine, idarubicin, and G-CSF (FLAG-IDA). Twenty-four (53%) patients achieved complete remission (CR), and five (11%) partial remission. Infection predominantly with pulmonary involvement was the most common regimen-related toxicity. Mucositis (15 patients) and pulmonary toxicity (19 patients) were the most frequently observed non-hematologic side effects. There were four early deaths and 12 patients presented with resistant disease. Overall survival (OS) at 12 months was 40%. The FLAG-IDA regimen shows evident antileukemic activity in patients with high-risk myeloid malignancies with acceptable toxicity.
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PMID:FLAG-IDA regimen (fludarabine, cytarabine, idarubicin and G-CSF) in the treatment of patients with high-risk myeloid malignancies. 1219 67

We describe here the development of a murine system for the identification of genes involved in myelomonocytic neoplasms. Transgenic C57BL/6J mice expressing SV40 early region under a myelomonocytic promoter develop histiocytic sarcomas with a latency of 167 days. We used retroviral proviral tagging to accelerate tumorigenesis and to uncover genetic changes that contribute to tumor development. Infection of transgenic mice with Friend murine leukemia virus (F-MuLV) shortened the latency of morbidity to 103 days (P< 0.001); this was associated with clonal proviral integrations in tumor DNA. As expected for F-MuLV, proviral insertions occurred at Fli1 in both transgenic and nontransgenic tumors. Four insertions were found at a novel locus, termed Fim4, on chromosome 6. This region is syntenic to human 7q32, a region that is commonly deleted in human myelodysplastic syndrome and acute myeloid leukemia. A murine BAC containing Fim4 was sequenced and analyzed, and while there was significant human-mouse homology in the area of the insertions, no candidate gene has been identified. Thus we have established a system to identify genes involved in myelomonocytic tumors, and have used it to identify Fim4, a new common site of proviral insertion. Study of this locus may provide insight into genes involved in AML-associated 7q32 deletions in humans.
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PMID:F-MuLV acceleration of myelomonocytic tumorigenesis in SV40 large T antigen transgenic mice is accompanied by retroviral insertion at Fli1 and a novel locus, Fim4. 1220 Jun 99

In this report we analyse the risk factors, the clinical characteristics and outcome of patients with myelodysplastic syndrome (MDS) who developed an Invasive Fungi Infection (IFI). This was a multicentric study involving 14 Italian Haematological Divisions during a 10-year period whose object was to identify the characteristics of patients with this infection. The study recorded 391 consecutive documented IFI, 12 of which (3%) occurred in MDS patients, from 5 of the participating centres. The primary localization of infection was lung in 10 cases and skin and paranasal sinus in 1 case each. Ten patients died at the end of follow up. The death was mainly attributable to IFI progression in nine of them. The factors that appeared related to an unfavourable outcome were intensive chemotherapy within 30 days before IFI diagnosis, presence of multiple localization at chest X-ray in patients with isolated pulmonary IFI and multiple sites of infection.
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PMID:Filamentous fungi infection in patients with myelodysplastic syndrome. A report of twelve cases. 1238 23

In the last decade, a growing number of patients with pneumonia, caused by unusual gram positive rods have been observed. Mostly, the patients had been infected as a consequence of impaired immunity. In some cases, bioterrorist activities may also induce pneumonia by gram positive rods (B. anthracis). In order to bring these organisms to the attention of the medical community, we present three clinical cases and describe six species of gram positive rods, known to provoke this kind of pneumonias. Case 1 was a 84 years old patient with impaired lung function. He was suspicious of tuberculosis (Tbc). Nocardia spec. was isolated. Case 2 was an alcoholic of 46 years with pneumonia. Reactivation of Tbc was suspected. Actinomadura madurae has been isolated. Case 3 was a patient of 58 years with myelodysplastic syndrome (MDS) and pneumonia. N. asteroides was isolated. All patients shared impaired immunity (age, alcoholism, MDS) with impaired lung functions; Tbc had been suspected (Case 1 + 2). Infection by A. madurae was contained by Clindamycin. Therapy of Nocardia with Moxifloxacin (Case 1) or Bactrim (Case 3) was only partly effective. In the appendix, six species of gram positive rods which are known to cause pneumonia, are summarized (Nocardia, Actinomyceta, Actinomadura, Rhodococcus, Corynebacterium and Bacillus).
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PMID:[Unusual gram positive rods, causing pneumonia]. 1278 78


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