Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In an open study, 42 venous Port-A-Cath systems (PAC) were implanted in 40 patients with AML (12), ALL/AUL (11), NHL with bone marrow infiltration (8), Hodgkin's lymphoma (3), solid tumors (5) and severe aplastic anemia (1). Mean duration of system use was 212 days. The cumulated duration of use of all systems was 8.883 days. 1,627 blood samples were taken from the PAC. Blood sampling was possible on 8,696 of 8,883 days of cumulated access (98%). A total of 522 blood transfusions were administrated. Fifty-two episodes of neutropenia (granulocyte counts less than 0.5 x 10(9)/l) with a mean duration of 17 days were observed in the group of the 23 patients with acute leukemias. A total of 25 complications were registered. The incidence was 2.8/1,000 days of access. Twelve complications were regarded as severe. Venous thrombosis was observed in 3 cases. In addition, there were 2 disruptions of the catheter, 1 disconnection, 1 looping and 4 local infections. The rate of systemic infection could not be accurately estimated because the catheter was always left in place and antibiotic treatment was started immediately in case of fever with or without bacteriemia. The overall rate of catheter-related complications in patients with acute leukemia was not higher than in patients with solid tumors.
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PMID:Use of a fully implantable drug delivery system in the treatment of acute leukemias and disseminated lymphomas. 224 62

There were 100 patients with pancytopenia notified to the Swedish part of an international study of aplastic anemia (Aa) (1983-1986). Aa was the cause in 16 patients and 50 patients had different conditions explaining their pancytopenia, whereas in 34 patients no obvious explanation was found at the time of discovery of their pancytopenia. A follow-up study in 1987 comprised the patients with Aa (n = 16) and the patients with uncharacterized pancytopenia (n = 34.3 additional patients had then been diagnosed as having Aa whereas 3 patients had a diagnosis of a low-grade NHL and 8 patients a diagnosis of MDS. No (or an inadequate) bone marrow sample had been taken in 9 of the 34 patients. This study shows that pancytopenia may be found in many serious conditions. Patients with pancytopenia should be carefully followed as Aa can develop slowly and as the MDS diagnosis can be difficult to establish at an early stage.
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PMID:Diagnoses in patients with severe pancytopenia suspected of having aplastic anemia. 237 59

The incidence of aplastic anemia appears to be relatively high in some parts of the world, including Pakistan. Since some of the etiological factors are shared by aplastic anemia and the preleukemic syndrome, there is a strong possibility that a proportion of cases of aplastic anemia may in fact be preleukemia. The study of chromosomes offers a relatively easy method of detecting cases of preleukemia, because some chromosomal abnormalities are frequently observed in this condition. Chromosomal studies were carried out in peripheral blood cell cultures of 31 patients with otherwise typical aplastic anemia. Chromosomal abnormalities were detected in 7 (22.5%) cases. The most common chromosomal abnormality detected was trisomy 8, seen in four cases. Other abnormalities detected were 22q-, t(14;22) and t(15;21), in one case each. These abnormalities have been found to be associated with AML, MDS, ALL, and NHL as well. We hypothesize that a proportion of cases of otherwise typical aplastic anemia may in fact be due to a leukemic process in evolution.
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PMID:Aplastic anemia or aplastic preleukemic syndrome? 943 74

From September 1982 to August 1997, 767 bone marrow or peripheral blood stem cell transplants have been performed at the Health Sciences Center in Oklahoma. Five hundred and two (502) autologous transplants (AutoTX) preceded by high-dose myeloablative therapy were performed for breast cancer (BC, 36%), non-Hodgkin's lymphomas (NHL, 24%), Hodgkin's disease (HD, 10%), acute myeloid leukemia (AML, 8%), testicular cancer (TC, 4%), multiple myeloma (MM, 2%) and other malignancies (16%). Two hundred and sixty-five (265) allogeneic marrow transplants (AlloTX) (related, unrelated) were carried out in chronic myeloid leukemia (CML, 30%), AML (23%), acute lymphoid leukemia (ALL, 14%), myelodysplastic syndrome (MDS, 9%), severe aplastic anemia (SAA, 8%), and other diseases (14%). Compared between 1980s to 1990s, 100-day mortality rates have decreased from 28% to 5% for AutoTX and from 40% to 25% for AlloTX. In the AutoTX setting, major changes included the routine use of growth factors post-transplant and the switch from bone marrow to growth factor-mobilized peripheral blood as a source of stem cells over the last five years. In the AlloTX setting, improvements in recognition and control of cytomegalovirus and Candida organisms, the selective use of growth factors and screened blood products, and better selection of unrelated donors using DNA-based techniques of HLA-matching have contributed to reduce early mortality from infection and primary graft failure. The five-year survival outcomes are comparable to those reported in registry data from the International Bone Marrow Transplant Registry (IBMTR) and the National Marrow Donor Program (NMDP).
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PMID:Marrow and stem cell transplantation in Oklahoma: fifteen years of experience and results. 976 68

HCV infection may affect not only the liver but also various nonhepatic tissues. This paper presents current information on association between HCV infection and haematological disorders. The pathogenic role of HCV in hepatitis-associated aplastic anaemia development has not been confirmed. The thrombocytopenia has been observed more frequently during chronic hepatitis C than during infections with other hepatotropic viruses. This disorder may be associated with antiplatelet autoantibodies production. However the most common haematological complication of HCV infection is mixed cryoglobulinemia (MC), observed in 40-50% of patients. In some subjects non-Hodgkin B cell lymphoma (B-NHL) may evolve from MC, but it is also reported in acryoglobulinemic HCV infected patients. The frequency of HCV infection in population of patients with B-NHL exceeds 20% in some countries and it is significantly higher than for other lymphoproliferative disorders. There are also data suggesting that HCV may play a role in MALT lymphoma development, too. The observed disorders are explained by HCV lymphotropism and direct or indirect influence of continuous antigenic stimulation by replicating virus on lymphatic system. The paper presents also beneficial results of interferon treatment in patients with HCV-related MC or B-NHL. The authors show that haematological syndromes should be taken under account in diagnostics of hepatitis C patients and interferon treatment should be administered as soon as possible when HCV related cryoglobulinaemia is diagnosed.
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PMID:[Hematologic syndromes in hepatitis C virus infection]. 1129 18

Hematopoietic colony-stimulating factors have been introduced into clinical practice as additional supportive measures to reduce infectious complications associated with congenital or acquired neutropenia. Over the past decade, we have begun to appreciate the subtler aspects of the proper use of G-CSF and GM-CSF, identifying appropriate indications and contraindications. In the course of evaluating the corpus of studies, a set of formal recommendations have been propagated for the judicious use of these expensive growth factors. To prevent serious infection, the use of G-CSF or GM-CSF is recommended in a subset of pediatric cancer patients shortly after having received chemotherapy or a form of a marrow transplant. Children with highly intensive chemotherapy (e.g., children with high risk ALL or NHL) seem to benefit from hematopoietic growth factors whereas this is still unclear for children undergoing therapy for solid tumors. An exciting development is the use of G-CSF and GM-CSF to mobilize peripheral-blood progenitor cells for autologous or allogeneic transplantation. In pediatric patients with hematological diseases, there are only few data on the use of hematopoietic growth factors in children with myelodysplastic syndrome. Experts recommend the early administration of G-CSF in children with very severe aplastic anemia. The use of G-CSF is also recommended in children with severe chronic neutropenia, but these patients have to be monitored regularly for cytogenetic abnormalities. No larger study has shown a clinical benefit of hematopoietic growth factor in preterm or term infants. Future studies in pediatric patients are clearly warranted to address several issues. Prospective clinical trials are still needed to define specific treatment groups who can benefit from growth factor support. In this regard, efforts must be directed at better defining the endpoints and in particular assigning value to reduction in treatment of possible infectious complications, such as days in hospital, antibiotic usage and costs. In addition, randomized studies are required to evaluate the proper dosage and duration of therapy, which most likely will vary between groups of patients. In addition, combinations of different growth factors have to be tested, particularly if ex vivo expansion and the storage of hematopoietic stem cells are to be utilized in a wider spectrum of patients.
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PMID:[Hematopoietic growth factors in prophylaxis and therapy of infections complications in children with neutropenia]. 1152 56

We assessed the prognostic importance of the platelet count 100 days post transplant of 107 consecutive patients receiving ablative allogeneic bone marrow transplant (BMT) between 7/96 and 12/00 who survived at least 100 days. Diagnoses included AML (n=36), chronic myelogenous leukemia (n=27), NHL (n=14), ALL (n=16), MDS (n=9), aplastic anemia (n=3), and one Hodgkin's disease and myelofibrosis each. In total, 64% were in remission or in chronic phase or had aplastic anemia (good risk), and 36% had active disease at the time of transplant (bad risk). In all, 70% were matched sibling transplants and 30% were matched unrelated donor transplants. The mean follow-up for the patients remaining alive is 48 months. Survival was powerfully influenced by the 100-day platelet count: 4-year survival was 19% for patients with a platelet count <30 x 10(9)/l; 41% for patients with a platelet count of 30-50; and 72% for those with a platelet count >50 (P<0.001; log-rank test). In a multivariable analysis, the most powerful risk factors for mortality after allogeneic BMT were low 100-day platelet count (P<0.001) and bad risk disease (P=0.009). We conclude that the platelet count 100 days post transplant is a powerful predictor of overall survival.
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PMID:Prognostic importance of the platelet count 100 days post allogeneic bone marrow transplant. 1468 14

X-linked lymphoproliferative syndrome (XLP) is caused by mutations in SH2D1A, and is associated with overwhelming infectious mononucleosis, aplastic anemia, hypogammaglobulinemia, and B-cell lymphomas. However, the frequency of SH2D1A mutations in males who present with B NHL is unknown. Five cases of XLP were diagnosed among 158 males presenting with B NHL (approximately 3.2%). Four of the patients had two episodes of B NHL and one had a single episode of B NHL followed by aggressive infectious mononucleosis. Prospective screening for XLP in males with B-cell lymphoma at the time of initial diagnosis should be considered.
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PMID:Frequent mutations in SH2D1A (XLP) in males presenting with high-grade mature B-cell neoplasms. 2358 80