Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We analysed a large homogeneous group of 14,403 patients transplanted for early leukaemia from an HLA-identical sibling and reported to the EBMT in four time cohorts: 1980-1989 (24%), 1990-1994 (26%), 1995-1998 (30%) and 1999-2001 (20%). We focused on death from infection. End points were survival, death from relapse and transplant-related mortality (TRM), which was subdivided into death from graft-versus-host disease (GvHD) (1315 patients; 25% of deaths), infection (597 patients; 11% of deaths) or 'other' causes (1875 patients; 34% of deaths). Survival increased from 52% at 5 years in the first to 62% in the third cohort (P<0.05) and TRM decreased from 36 to 26% (P<0.05) due to a reduction in death from infection (P<0.001). GvHD, 'other' causes and relapse did not improve. The relative proportions of bacteria (217 patients; 36%), viruses (183 patients; 31%), fungi (166 patients; 28%) or parasites (32 patients; 5%) as cause of infectious death (cumulative incidence of death at 5 years 1.8, 1.6, 1.4 and > or = 0.3%, respectively) and median time to death from infections (3 months (range 0-158 months)) did not change. Death from infections has been reduced significantly, but it still represents an ongoing risk after HSCT and draws attention to the time beyond the initial period of neutropenia.
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PMID:Cause of death after allogeneic haematopoietic stem cell transplantation (HSCT) in early leukaemias: an EBMT analysis of lethal infectious complications and changes over calendar time. 1615 26

The diagnosis of acute graft versus host disease (aGVHD) following liver transplantation can be difficult, since many of the clinical signs can be caused by drug reactions or viral infections. To establish criteria for the persistence of donor T-cells versus engraftment, we measured donor T-cells by short tandem repeat (STR) assays in 49 liver transplant patients for 8 or more weeks post-transplant. Donor CD3+ T-cells were detected in 38 of 49 patients, on POD 2 with a mean level of 5%. The top of the 99% confidence interval for weeks 1, 2, 3, 4 and 8 were 11, 6, 3, 2 and 3%. Donor CD8+ T-cells were measured in eight patients. The level of CD8+ T-cells was much less than that for CD3+ T-cells, except in two cases of apparent aGVHD. One patient developed severe aGVHD with donor T-cells as high as 84%. The other had 10% donor T-cells for more than 16 weeks associated with fever and neutropenia. We tested the sensitivity of PCR-ssp typing of HLA DR/DQ for donor T-cells. At least one donor type was detected in all samples with 1% or more donor DNA. Thus, higher levels of donor T-cell chimerism, particularly with a high proportion of CD8+ T-cells, strongly supports a diagnosis of aGVHD.
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PMID:Acute graft versus host disease after liver transplantation: patterns of lymphocyte chimerism. 1630 12

Hematopoietic cell transplantation (HCT) following nonmyeloablative conditioning (NMSCT) may be associated with a reduced risk of infection compared to standard allogeneic HCT. We retrospectively analyzed incidence and risk factors of infection in 62 patients undergoing NMSCT with low-dose TBI +/- fludarabine and postgrafting CsA and MMF. The proportion of patients with any infection was 77%, but the majority of infectious events occurred beyond day 30. Donor other than sibling, older age, early disease and male gender were significant risk factors. The incidence of bacteremia was 55% at 1 year and the number of bacteremic episodes was 0.9 per patient (0.08 before day 30). The risk of bacteremia increased with older age and the use of a donor other than an HLA-identical sibling, but not with neutropenia. The incidence of infections other than bacteremia correlated with the use of corticosteroids. The risk of CMV infection increased with high-risk CMV serology, and risk of CMV disease with high-risk CMV serology, older age, first transplantation and a diagnosis of lymphoma. In conclusion, after NMSCT, infections are not frequent in the first 30 days post transplant but careful long-term monitoring is necessary thereafter.
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PMID:Infections after allogeneic hematopoietic stem cell transplantation with a nonmyeloablative conditioning regimen. 1641

We evaluated the occurrence of severe infections in 192 consecutive adult recipients of volunteer unrelated donor allogeneic hematopoietic stem cell transplants, with a detailed analysis of severe infections after receipt of cord blood transplants (CBTs; n = 48) or bone marrow transplants (BMTs)/peripheral blood stem cell transplants (PBSCTs; n = 144). At a 3-year median follow-up, CBT recipients had a higher risk of developing any severe infection (85% versus 69% in BMT/PBSCT recipients, P < .01). CBT recipients had a higher incidence of severe bacterial infections before day +100, but at 3 years the risks of these and other infections were similar in the CBT and BMT/PBSCT groups. In addition, the 100-day and 3-year incidences of infection-related mortality (IRM) did not differ between groups (P = .2 and .5, respectively). In multivariate analysis, the most significant risk factor for IRM in all 192 patients was monocytopenia (.2 x 10(9)/L). In CBT recipients, only neutropenia (.2 x 10(9)/L) on day +30 and low nucleated cell dose infusion (< 2 x 10(7)/kg) showed a trend for increased IRM (P = .05 in both cases). Stem cell source had no effect on day +100 or 3-year nonrelapse mortality (NRM), cytomegalovirus infection, cytomegalovirus disease (7% versus 6%), or overall survival (36% versus 39%, respectively). The number of mismatches in HLA (A, B, and DRB1) had no effect on any outcome in CBT recipients. In contrast, in the BMT/PBSCT group, the presence of any mismatch by low or high-resolution HLA typing (A, B, C, and DRB1) increased NRM and decreased overall survival (P < .01). IRM was the primary or secondary cause of death in 61% and 59% of CBT and BMT/PBSCT recipients who died, respectively. Our results confirm the relevance of severe infectious complications as source of severe morbidity and NRM after volunteer unrelated donor hematopoietic stem cell transplantation in adults, but suggest that CBT recipients have a similar risk of dying from an infection if an accurate selection of a cord blood unit is done.
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PMID:Severe infections after unrelated donor allogeneic hematopoietic stem cell transplantation in adults: comparison of cord blood transplantation with peripheral blood and bone marrow transplantation. 1678 63

Neonatal alloimmune neutropenia (NAN) is a disease that can cause severe and prolonged neutropenia in neonates. However, no report is available on the incidence of granulocyte antibody in neonates, the target antigen of this antibody, and the estimated incidence of NAN in Korea. Among a total of 856 neonates admitted to a neonatal intensive care unit (NICU) over a five year period, a total of 105 neonates with neutropenia were enrolled in this study. Positive reactions were observed in the sera of six neonates (5.7%, 6/105) by mixed passive hemagglutination assay (MPHA). To confirm the presence of NAN, MPHA and granulocyte antigen typing (HNA-1a, -1b, -2a, -4a, and -5a) were performed on neonatal and maternal blood. To differentiate granulocyte antibody and HLA antibody, MPHA was also performed using HLA antibody adsorbed serum. We confirmed three cases (2.9%, 3/105) of NAN among neonates with neutropenia in which granulocyte antibody specificities (two anti-HNA-1b and one anti-HNA-1a) and fetomaternal granulocyte antigen mismatches were identified. In this study, the estimated incidence of NAN was 0.35% (3/856) among neonates admitted to NICUs in Korea.
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PMID:Granulocyte antibodies in Korean neonates with neutropenia. 1689 4

Although aspergillosis remains the most common mould infection in patients with haematologic malignancies, disseminated Fusarium infection is an emerging problem with a poor prognosis in this patient population. The treatment options are limited due to relative resistance of the fungus to standard antifungals. We present a patient with acute lymphoblastic leukaemia successfully treated with AmBisome for a disseminated Fusarium solani infection that did not respond to first line treatment with voriconazole. Despite the fact that he received additional myelosuppressive chemotherapy and underwent two stem cell transplantations from HLA mismatched donors the Fusarium infection did not recur during the subsequent phases of neutropenia. The clinical presentation, diagnosis, prognosis and therapeutic options of fusariosis in immunocompromised patients are briefly discussed.
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PMID:A case of fusariosis in an immunocompromised patient successfully treated with liposomal amphotericin B. 1691 66

Omenn syndrome is a severe combined immunodeficiency characterized by erythroderma, hepatosplenomegaly, lymphadenopathy and failure to thrive, with activated oligoclonal T lymphocytes and an absence of circulating B cells.A 3 day-old boy presented with a congenital erythroderma. Investigations revealed a marked neutropenia and lymphopenia and the absence of a thymus. Genetic studies showed RAG 1 mutations. He was successfully treated with an HLA identical bone marrow transplantation. Omenn syndrome is a rare severe combined immunodeficiency. Most cases are due to mutations in the RAG genes with autosomal recessive transmission. Our observation is original because of an incomplete clinical presentation. During the course of the disease, the child had no failure to thrive, no organomegaly and no recurrent infection. Immunodeficiency must be excluded in every case of neonatal erythroderma and an immunological assessment should be performed without delay.
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PMID:Omenn syndrome: a rare case of neonatal erythroderma. 1733 97

PNP deficiency is an autosomal recessive metabolic disorder characterized by severe combined immunodeficiency, autoimmune hemolytic anemia, and by a complex of neurologic manifestations including ataxia, developmental delay, and spasticity. PNP protein catalyzes the phosphorolysis of deoxyinosine and deoxyguanosine. It is found in most tissues of the body but is expressed at the highest levels in lymphoid tissues. This tissue distribution explains why the lymphoid system is predominantly affected in PNP deficiency. We describe a five-yr-old boy with muscular hypertonia, impaired growth, autoimmune hemolytic anemia, and neutropenia who underwent HSCT from his HLA-identical sister. One yr post-HSCT, the boy developed normal immunological functions, and his neurological status improved.
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PMID:Successful HLA-identical hematopoietic stem cell transplantation in a patient with purine nucleoside phosphorylase deficiency. 1791 Jun 61

We describe a recipient of combined kidney and hematopoietic-cell transplants from an HLA-matched donor. A post-transplantation conditioning regimen of total lymphoid irradiation and antithymocyte globulin allowed engraftment of the donor's hematopoietic cells. The patient had persistent mixed chimerism, and the function of the kidney allograft has been normal for more than 28 months since discontinuation of all immunosuppressive drugs. Adverse events requiring hospitalization were limited to a 2-day episode of fever with neutropenia. The patient has had neither rejection episodes nor clinical manifestations of graft-versus-host disease.
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PMID:Tolerance and chimerism after renal and hematopoietic-cell transplantation. 1821 63

Fifty-three patients with hematological malignancies who underwent Allo-SCT from HLA-identical siblings were randomly assigned to receive glutamine-enriched parenteral nutrition-PN (GlPN, n=27) or standard PN (PN, n=26), in isonitrogenous solutions. Deaths (D+100 and D+180), infections, acute GVHD, length of stay, time of neutropenia and intestinal permeability (IP) were studied. Ages, gender, diagnosis, disease status and treatment variables were equally distributed between groups. Survival on D+180 was increased in GlPN (74%) vs PN (46%), P=0.03 (log-rank), as on D+100 (P=0.05). Most deaths occurred before D+100, especially in PN (10/26, 39%) vs GlPN (4/27, 15%). GVHD was the most frequent cause of death (8/21, 38%), especially in PN (n=6, five before D+100). Other outcomes were not affected. IP was affected on admission, was not affected by glutamine enrichment, but consistently worsened throughout the study. Results showed that GlPN was efficacious in increasing short-term survival after Allo-SCT. Benefits of glutamine seem to be independent of mucosal protection, as IP was not affected by its use. A trend to a lower incidence of GVHD deaths may suggest an immunomodulatory role of glutamine.
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PMID:Efficacy of glutamine-supplemented parenteral nutrition on short-term survival following allo-SCT: a randomized study. 1831 56


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