Biomedical subjects

L J Bosch

Publications and source records attributed to L J Bosch.

5 recordsLinked to original sources

PubMed2000

Disappearance of factor VIII autoantibodies preceding autoimmune haemolytic anaemia.

We describe a previously healthy woman who at the age of 44 years developed a factor VIII inhibitor, that over the years increased to a maximum level of 3600 Bethesda units (BU) mL(-1) in 1978. The epitope specificity of the factor VIII inhibitor was investigated and antibodies directed against the A2 and C2 domains of factor VIII were detected. The majority of these antibodies were of subclass IgG4. Over the years, the inhibitor titre gradually decreased and in 1989, the inhibitor could no longer be detected. Shortly after, the patient developed autoimmune haemolytic anaemia. A possible link between the disappearance of factor VIII inhibitors and the development of other autoantibodies may be explained by concomitant development of anti-idiotypic antibodies that neutralize the activity of factor VIII inhibitors. We were unable to detect anti-idiotypic antibodies, which could explain the decline in factor VIII inhibitor titre in this patient.

Aged↗

PubMed1994

Congenital hypoplastic anemia: another example of autosomal dominant transmission.

Congenital hypoplastic anemia (CHA) or Blackfan-Diamond anemia (BDA) is a rare congenital abnormality of erythropoiesis characterized by normochromic, macrocytic anemia presenting in infancy or early childhood. Associated phenotypic abnormalities such as triphalangeal thumbs and cleft lip and/or palate are found in 70% of cases. Although most cases are sporadic, several reports suggest either autosomal dominant or autosomal recessive inheritance. We report on a 3 generation family with autosomal dominant inheritance of CHA.

Adult↗

PubMed1992

Hodgkin disease in the elderly. A registry-based analysis.

Limited data regarding the cause of the age-related decrease in survival times in patients with Hodgkin disease are available. In a retrospective study of a nonselected population of patients with Hodgkin disease, the authors evaluated which factors contributed to the age-related prognostic effect in this disease. The survival curves of 182 patients were compared, and survival time was found to decrease markedly after the age of 50 years. Differences in disease characteristics between older and younger patients were small and not statistically significant. Significantly fewer older patients received adequate treatment (34% versus 2%), and they were less likely to have complete disease remission (61% versus 90%). However, the relapse-free survival time of patients with complete disease remission was not significantly different from that of younger patients, 50% of all patients being free of disease after 10 years. Intercurrent disease did not appear to be responsible for decreased survival times in the elderly (32% versus 26%). The authors conclude that the inability to give adequate treatment seems to be the major determinant of the poorer overall survival time of older patients with Hodgkin disease.

Age Factors↗

PubMed1989

Grades of nodular sclerosis (NSI-NSII) in Hodgkin's disease. Are they of independent prognostic value?

Subclassification of the nodular sclerosis (NS) type of Hodgkin's disease in Grade 1 and 2 was reported for the first time by the British National Lymphoma Investigation (BNLI). Three groups, the BNLI, Gärtner et al. and the current authors, found clearly different survival rates between Grade 1 and 2 NS patients. The authors studied retrospectively if this NS grading has an independent prognostic value in 90 NS patients, diagnosed in ten hospitals in the southeastern part of the Netherlands (1972-1983). In this study there is no significant difference in sex, age, B-symptoms, erythrocyte sedimentation rate (ESR), stage, positive laparotomy, involvement of mediastinum or spleen, lymphocyte count, and percentage of complete remissions between the NS subgroups. Multivariate analysis suggests that the subclassification of NS in Grades 1 and 2 is a prognostic factor in survival independent of age, stage and ESR. This finding and the high relative frequency of NS makes application of this NS subdivision probably clinically useful to identify patients for a risk-adapted therapy.

Adult↗

PubMed1978

Factor VIII inhibitor postpartum.

Acquired factor VIII deficiency in women postpartum due to a factor VIII inhibitor is rare and the etiology is unknown. In this study a case report and a review of the literature are given. The haemorrhagic diathesis resembles classic haemophilia, with the exception that ecchymoses and tissue bleeding occur more frequently. The potency of the inhibitor may vary from weak to strong and the inactivation of factor VIII coagulant activity (factor VIII-C) by the inhibitor is of a non-linear type. Severe bleeding has been fatal in a few cases, but factor VIII concentrate substitution has usually been successful without anamnestic response of inhibitor activity. There is no convincing evidence that immunosuppression is effective, also because the natural history of the disease is characterised by a spontaneous disappearance of the factor VIII-C inhibitor. Treatment of bleeding symptoms with factor VIII concentrate should therefore not be reserved for life threatening haemorrhages only.

Adult↗