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Biomedical subjects

J Vossen

Publications and source records attributed to J Vossen.

29 records · Page 2Linked to original sources
PubMed1990

Treatment with marrow transplantation or immunosuppression of childhood acquired severe aplastic anemia: a report from the EBMT SAA Working Party.

A total of 304 children under the age of 15 years with acquired severe aplastic anemia (SAA) received immunosuppressive therapy (IS) (n = 133) or a matched bone marrow transplant (BMT) (n = 171). The projected 10-year survival is 48% and 63% respectively (p = 0.002). Results following BMT have improved considerably over the years from 49% in 1970-80, to 70% in 1981-83 (p = 0.002) and to 81% between 1984-88 (p = 0.08). Other favorable prognostic factors are the use of cyclosporin A (p = 0.004), no previous therapy (p = 0.006) and early BMT (p = 0.009). In multivariate analysis only the year of treatment proved significant (p = 0.02). In contrast, results of IS are greatly dependent on the severity of pre-treatment neutropenia with survival of 56% versus 37% for neutrophils more or less than 0.2 x 10(9)/l (p = 0.003). Poor survival was associated in univariate analysis with female sex (43%), post-hepatitis SAA (37%), children not receiving androgens (38%) and patients younger than 5 years (35%), especially if associated with a low neutrophil count (11%). In multivariate analysis only the degree of neutropenia proved significant (p = 0.005). These results suggest that IS is a satisfactory alternative therapy for children with moderately SAA in the absence of an HLA-identical sibling, although BMT remains the treatment of choice. In children under 5 years with very SAA, results with IS are so poor that a search for an unrelated matched donor is justified as early as possible.

Actuarial Analysis↗
PubMed1989

Monoclonal gammopathies in children.

Over a 10-year period sera of 4000 pediatric patients were subjected to agar gel electrophoresis and immunoelectrophoresis. Retrospective examination of the electrophoresis patterns indicated that single or multiple homogeneous immunoglobulin components were present in sera of 155 children (3.9%). They were most frequently found in patients suffering from primary and secondary immunodeficiency diseases, hematological malignancies, autoimmune diseases, and severe aplastic anemia. Follow-up analysis revealed that most of these monoclonal gammopathies were transient. The monoclonal gammopathies in the serum of 79 patients were identified by immunoblotting for class and light-chain isotypes. A marked absence of IgA monoclonal gammopathies and a predominance of monoclonal gammopathies of the lambda light-chain isotype were found. Most of the B-cell mono- or oligoclonal proliferations in children can probably develop due to a disturbance in the regulatory T-cell function.

Child↗
PubMed1989

Allogeneic bone marrow transplantation for secondary leukaemia and myelodysplastic syndromes. Leukaemia Working Party of the European Bone Marrow Transplantation Group (EBMTG).

This retrospective survey of the EBMT Leukaemia Working Parking describes 78 patients with myelodysplasia (MDS) or secondary acute myelogenous leukaemia (sAML) who were given an allogeneic bone marrow transplant (BMT). The status of underlying disease at the time of transplantation was prognostic for the two-year disease-free survival which was 60% for patients transplanted in complete remission. Similar results were obtained for those with less advanced MDS (50-64%) who had not received any prior intensive chemotherapy. The results were significantly less favourable for those with more advanced disease who only partially responded to prior intensive chemotherapy (18%) while none of those who either relapsed or were resistant to chemotherapy survived. Allogeneic BMT can therefore be considered as curative treatment for patients with MDS. Patients with sAML who have a histocompatible donor should be given chemotherapy intensive enough to induce complete remission. If this is achieved these individuals have a prognosis comparable to those with de novo AML in first remission after BMT.

Adolescent↗
PubMed1989

Allogeneic bone marrow transplantation (BMT) for acquired severe aplastic anaemia (SAA) in children.

The SAA Registry of the EBMT now contains data on 171 children younger than 15 years of age with acquired SAA and undergoing BMT between 1970 and 1988. The overall actuarial survival is 63% at 10 years. In a multivariate Cox analysis, the year of transplant was the most important prognostic factor with a significant advantage for children grafted in 1984-88 (81%) vs 1981-83 (67%) and 1970-80 (41%) (p = 0.02). Cyclosporine A given for GVHD prophylaxis, no treatment before transplant and an interval less than 90 days from diagnosis to BMT were all favourable variables in univariate analysis. As regard to transplant procedures, the better results were obtained using Cyclophosphamide and Cyclosporine A (78%) followed by Cyclophosphamide plus irradiation plus Cyclosporine A (77%). Sex, etiology and the severity of the aplasia had no impact on survival in both uni and multivariate analysis.

Adolescent↗
PubMed1986

Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985.

In this retrospective analysis of allogeneic bone-marrow transplantation (BMT) carried out between 1969 and 1985 at fourteen European centres in 162 patients with sixteen different types of inherited immunodeficiencies and osteopetrosis, the overall survival with functional grafts was 51.7% (85 patients), with a minimum follow-up of 5 months. In patients with severe combined immunodeficiency HLA-matched (n = 41) and T-cell-depleted HLA-mismatched BMT (n = 46) resulted in 68% and 57% disease-free survival, respectively; after HLA-mismatched transplants, older age (greater than 6 months) and adenosine-deaminase deficiency resulted in poorer survival. Eight other lethal immunodeficiencies, including profound T-cell deficiencies, Wiskott-Aldrich syndrome, Kostmann syndrome, LFA-1/CR 3/p150,95 deficiency, and Chediak-Higashi syndrome as well as malignant osteopetrosis, have been successfully treated by BMT. In this group, survival with functional graft was 47% with HLA-matched and 29% with T-cell-depleted HLA-mismatched BMT. Engraftment failure was the major complication in this group. Poorer prognosis was associated with older patients, profound T-cell deficiencies, and the degree of HLA incompatibility.

Adolescent↗
PubMed1984

Long-term observations after trephination with scleral flap in glaucoma with threatened point of fixation.

Follow-up examinations 2 months to 9 years after a filtering operation with scleral flap with successful pressure regulation could be carried out in 26 patients with 29 eyes in which a threat to the point of fixation due to glaucoma was present. In the majority of the cases, it was possible to stabilize visual acuity and visual field by the operation. In the remaining cases, there was a slow melting away of the residual visual field without correlation with lens or macular changes or underlying internal conditions.

Adult↗
PubMedDate not supplied

C-reactive protein in the management of children with fever after allogeneic bone marrow transplantation.

The value of C-reactive protein (CRP) determinations in the analysis of fever after allogeneic bone marrow transplantation (BMT) was studied prospectively by serial measurements of serum CRP levels during 30 BMT episodes in 28 children and adolescents. The treatments and procedures accompanying BMT did not elicit a significant CRP response. Forty-three febrile episodes were registered and analyzed, without previous knowledge of the results of CRP determinations. The incidence of bacterial infection and acute graft-versus-host disease (GvHD) was low, 8/30 and 5/30, respectively. Raised CRP levels occurred only once in association with GvHD. A CRP level higher than 50 mg/l was not sensitive as an indicator of bacterial infection (4/8). A CRP level below 50 mg/l in the presence of fever, however, excluded bacterial infection with a specificity of 86% and a negative predictive value of 88%. When timed properly and interpreted together with clinical and microbiological findings, CRP measurements can be a valuable aid in the management of fever after BMT, especially as a negative predictor.

Adolescent↗