| 1. |
|
|
| 2. |
|
|
| 3. |
|
|
| 4. |
- Mellgren, Karin, 1962, et al.
(författare)
-
Chimerism analysis in clinical practice and its relevance for the detection of graft rejection and malignant relapse in pediatric hematopoietic stem cell transplant patients
- 2015
-
Ingår i: Pediatric Transplantation. - : Wiley. - 1397-3142 .- 1399-3046. ; 19:7, s. 758-766
-
Tidskriftsartikel (refereegranskat)abstract
- Chimerism and clinical outcome data from 244 hematopoietic stem cell transplants in 218 children were retrospectively analyzed to assess their relevance for the detection of graft rejection and malignant relapse. Patients transplanted for a non-malignant disease had significantly higher proportions of residual recipient T cells in peripheral blood at one, three, and sixmonths compared with patients transplanted for malignant disease. Recipient T-cell levels were below 50% at onemonth after transplantation in most patients (129 of 152 transplants). Graft rejection occurred more frequently in the group of patients with high levels of recipient cells at onemonth (10 graft rejections in the 23 patients with recipient T cells >50% at onemonth as compared to seven graft rejections occurred in 129 patients with recipient T cells <50% (p<0.001). Multilineage chimerism data in 87 children with leukemia at one, three, and sixmonths after transplantation were not correlated with subsequent relapse of malignant disease. In conclusion, early analysis of lineage-specific chimerism in peripheral blood can be used to identify patients who are at high risk of graft rejection. However, the efficacy of early chimerism analysis for predicting leukemia relapse was limited.
|
|
| 5. |
- Riva, Roberto, et al.
(författare)
-
Patterns of psychological responses in parents of children that underwent stem cell transplantation
- 2014
-
Ingår i: Psycho-Oncology. - : Wiley. - 1057-9249 .- 1099-1611. ; 23:11, s. 1307-1313
-
Tidskriftsartikel (refereegranskat)abstract
- ObjectiveHematopoietic stem cell transplantation (HSCT) is curative in several life-threatening pediatric diseases but may affect children and their families inducing depression, anxiety, burnout symptoms, and post-traumatic stress symptoms, as well as post-traumatic growth (PTG). The aim of this study was to investigate the co-occurrence of different aspects of such responses in parents of children that had undergone HSCT. MethodsQuestionnaires were completed by 260 parents (146 mothers and 114 fathers) 11-198 months after HSCT: the Hospital Anxiety and Depression Scale, the Shirom-Melamed Burnout Questionnaire, the post-traumatic stress disorders checklist, civilian version, and the PTG inventory. Additional variables were also investigated: perceived support, time elapsed since HSCT, job stress, partner-relationship satisfaction, trauma appraisal, and the child's health problems. A hierarchical cluster analysis and a k-means cluster analysis were used to identify patterns of psychological responses. ResultsFour clusters of parents with different psychological responses were identified. One cluster (n=40) significantly differed from the other groups and reported levels of depression, anxiety, burnout symptoms, and post-traumatic stress symptoms above the cut-off. In contrast, another cluster (n=66) reported higher levels of PTG than the other groups did. ConclusionsThis study shows a subgroup of parents maintaining high levels of several aspects of distress years after HSCT. Differences between clusters might be explained by differences in perceived support, the child's health problems, job stress, and partner-relationship satisfaction.
|
|
| 6. |
- Ahlin, Anders, et al.
(författare)
-
Chronic granulomatous disease - haematopoietic stem cell transplantation versus conventional treatment.
- 2013
-
Ingår i: Acta paediatrica (Oslo, Norway : 1992). - : Wiley. - 1651-2227 .- 0803-5253. ; 102:11, s. 1087-1094
-
Tidskriftsartikel (refereegranskat)abstract
- Chronic granulomatous disease (CGD) is a rare X-linked or autosomal recessive primary immune deficiency characterized by recurrent, life-threatening bacterial and fungal infections. Mortality rates are high with conventional treatment. However, haematopoietic stem cell transplantation (HSCT) offers cure. Here, we compare the outcome of HSCT in 14 Swedish patients with CGD to that in 27 patients with CGD who were given conventional treatment.
|
|
| 7. |
|
|
| 8. |
- Borte, Stephan, et al.
(författare)
-
Neonatal screening for severe primary immunodeficiency diseases using high-throughput triplex real-time PCR.
- 2012
-
Ingår i: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 119:11, s. 2552-2555
-
Tidskriftsartikel (refereegranskat)abstract
- Severe combined immunodeficiency (SCID) and X-linked agammaglobulinemia (XLA) are inborn errors of immune function that require prompt diagnosis and treatment to prevent life-threatening infections. The lack of functional T- or B-lymphocytes in these diseases serves as a diagnostic criterion and can be applied to neonatal screening. A robust triplex PCR method for quantitation of T cell receptor- (TREC) and kappa-deleting recombination excision circles (KREC), using a single Guthrie card punch, was developed and validated in a cohort of 2.560 anonymized newborn screening cards and in 50 original stored Guthrie cards from patients diagnosed with SCID, XLA, Ataxia-telangiectasia (AT), Nijmegen-breakage-syndrome (NBS), Common variable immunodeficiency (CVID), Immunoglobulin-A-deficiency (IgAD), or X-linked Hyper-IgM-syndrome (X-HIGM). Simultaneous measurement of TREC and KREC copy numbers in Guthrie card samples readily identified patients with SCID, XLA, AT and NBS and thus facilitates effective newborn screening for severe immunodeficiency syndromes characterized by the absence of T or B cells.
|
|
| 9. |
- Borte, Stephan, et al.
(författare)
-
Newborn screening for severe T and B cell lymphopenia identifies a fraction of patients with Wiskott-Aldrich syndrome.
- 2014
-
Ingår i: Clinical immunology (Orlando, Fla.). - : Elsevier BV. - 1521-7035 .- 1521-6616. ; 155:1, s. 74-78
-
Tidskriftsartikel (refereegranskat)abstract
- The lack or marked reduction of recently formed T and B cells provides a basis for neonatal screening for severe combined immunodeficiencies (SCID) and X-linked agammaglobulinemia (XLA). Newborns with other conditions are also identified if a severe T or B cell lymphopenia is present at birth. We retrospectively analyzed Guthrie card samples from 11 children with Wiskott-Aldrich syndrome (WAS), a rare disease that requires early diagnosis and treatment, to determine whether combined T-cell receptor excision circle (TREC) and kappa-deleting recombination excision circle (KREC) screening could identify these patients. 4 of 11 patients showed markedly reduced TREC or KREC copy numbers in their DBS as compared to storage-time matched controls and prospectively screened Swedish and German newborns. No correlation was observed between the WAS gene mutations, the clinical severity/course and the result of the screening assay. A diagnosis of WAS should thus be considered in newborns with positive TREC or KREC screening results.
|
|
| 10. |
- El-Serafi, Ibrahim, et al.
(författare)
-
Reduced Risk of Sinusoidal Obstruction Syndrome of the Liver after Busulfan-Cyclophosphamide Conditioning Prior to Allogeneic Hematopoietic Stem Cell Transplantation
- 2020
-
Ingår i: Clinical and Translational Science. - : Wiley. - 1752-8054 .- 1752-8062. ; 13:2, s. 293-300
-
Tidskriftsartikel (refereegranskat)abstract
- The aim of this study is to evaluate the incidence of sinusoidal obstruction syndrome (SOS) of the liver and the clinical outcome after hematopoietic stem cell transplantation (HSCT) based on several modifications in our protocols. We retrospectively investigated 372 patients undergoing myeloablative conditioning with oral busulfan (Bu) and cyclophosphamide before allogeneic HSCT during 1990-2015. Patients' supportive care was changed in order to reduce the regimen-related toxicities. Norethisterone use was terminated in 1998, therapeutic drug monitoring of Bu was initiated in 2000, and the use of liver supportive drugs, such as ursodeoxycholic acid and N-acetyl-L-cysteine, were started in 2002 and 2009, respectively. In total, 26 patients (7.0%) developed SOS at a median of 19 days after transplantation. Of these 26 patients, 20 died at a median of 119 days after HSCT and 102 days after the diagnosis of SOS. The incidence of SOS decreased over time in accordance with the improvements in supportive care. The highest incidence of SOS was during 1995-1999 (16.2%) compared with 2.3% during 2010-2015. Overall survival for patients with SOS was 62%, 46%, and 27% at 100 days, 1 year, and 5 years after HSCT, respectively, compared with 92%, 77%, and 66% for those who did not develop SOS (P < 0.001). In conclusion, the incidence of SOS and related deaths were significantly decreased over the last years. Our institution pursues massive preventative and personalized measures for SOS. This strategy may also be applicable in other conditioning protocols in order to reduce the incidence of SOS and, hence, improve the clinical outcome.
|
|
