A nine-yr-old boy with EPP suffered from severe skin burns and liver failure caused by progressive cholestasis and fibrosis. OLT was performed without major complications. Four months following... Show moreA nine-yr-old boy with EPP suffered from severe skin burns and liver failure caused by progressive cholestasis and fibrosis. OLT was performed without major complications. Four months following liver transplantation he underwent parental haploidentical HSCT. The myeloablative conditioning regimen was relatively well tolerated and hematological engraftment was rapid (on day 10). Protoporphyrin concentrations returned to normal following HSCT. However, immune recovery was significantly delayed. Varicella zoster virus reactivation resulted in impaired vision, prolonged hospitalization and eventually in multiorgan failure and death. Sequential liver and haploidentical HSCT proved feasible though a high risk procedure in this EPP patient. The management of post-IST after these combined transplantations remains a challenge and needs to be further established. Show less
Introduction. Resection of pulmonary metastases has previously been reported to improve Outcome in high-grade osteosarcoma (OS) patients. Factors influencing Survival in OS patients with pulmonary... Show moreIntroduction. Resection of pulmonary metastases has previously been reported to improve Outcome in high-grade osteosarcoma (OS) patients. Factors influencing Survival in OS patients with pulmonary metastases are important for clinical decision making. Methods. All 88 OS patients with pulmonary metastases either at diagnosis or during follow-up treated at the Leiden University Medical Center between January 1, 1990 and January 1, 2008 under the age of 40 were included in this Study, including 79 cases of conventional, 8 cases of telangiectatic and 1 case of small cell OS. Results. In total, 56 of 88 patients with pulmonary metastases were treated by metastasectomy. Resectability Of pulmonary metastases was the main prognostic factor. In patients with primary non-metastatic OS, a longer relapse free interval to Pulmonary metastases was significantly associated with better survival (P=0.02). Independent risk factors determining worse survival after metastasectomy in multivariate analysis were male sex (P=0.05), higher number of pulmonary nodules (P=0.03), and non-necrotic metastases (P=0.04). Whether Surgery for recurrent pulmonary metastases was performed did not influence Survival. Histological Subtype of the primary tumor, histological response in the primary tumor after neoadjuvant chemotherapy, occurrence of local relapse, local resection or amputation of the primary tumor and age at diagnosis did not influence Outcome. Conclusion. This cohort of patients with detailed follow-up data enabled US to identify important risk factors determining Survival in OS patients with pulmonary metastases. We demonstrate that after repeated metastasectomies, a subset of patients can be cured. Pediatr Blood Cancer 2010;54:216-221. (C) 2009 Wiley-Liss, Inc. Show less
INTRODUCTION: Resection of pulmonary metastases has previously been reported to improve outcome in high-grade osteosarcoma (OS) patients. Factors influencing survival in OS patients with pulmonary... Show moreINTRODUCTION: Resection of pulmonary metastases has previously been reported to improve outcome in high-grade osteosarcoma (OS) patients. Factors influencing survival in OS patients with pulmonary metastases are important for clinical decision making. METHODS: All 88 OS patients with pulmonary metastases either at diagnosis or during follow-up treated at the Leiden University Medical Center between January 1, 1990 and January 1, 2008 under the age of 40 were included in this study, including 79 cases of conventional, 8 cases of telangiectatic and 1 case of small cell OS. RESULTS: In total, 56 of 88 patients with pulmonary metastases were treated by metastasectomy. Resectability of pulmonary metastases was the main prognostic factor. In patients with primary non-metastatic OS, a longer relapse free interval to pulmonary metastases was significantly associated with better survival (P = 0.02). Independent risk factors determining worse survival after metastasectomy in multivariate analysis were male sex (P = 0.05), higher number of pulmonary nodules (P = 0.03), and non-necrotic metastases (P = 0.04). Whether surgery for recurrent pulmonary metastases was performed did not influence survival. Histological subtype of the primary tumor, histological response in the primary tumor after neo-adjuvant chemotherapy, occurrence of local relapse, local resection or amputation of the primary tumor and age at diagnosis did not influence outcome. CONCLUSION: This cohort of patients with detailed follow-up data enabled us to identify important risk factors determining survival in OS patients with pulmonary metastases. We demonstrate that after repeated metastasectomies, a subset of patients can be cured. Show less
Lankester, A.C.; Ball, L.M.; Lang, P.; Handgretinger, R. 2010
Immunotherapy in the context of hematopoietic stem cell transplantation has been dominated for many years by T-cell- and dendritic-cell-based treatment modalities. During the last decade, insight... Show moreImmunotherapy in the context of hematopoietic stem cell transplantation has been dominated for many years by T-cell- and dendritic-cell-based treatment modalities. During the last decade, insight into the biology of natural killer (NK) cells and mesenchymal stromal cells (MSC) has rapidly increased and resulted in NK- and MSC-based therapeutic strategies in clinical practice. This article reviews current knowledge of the biology and clinical aspects of NK cells and MSC. Show less
The past 25 years have seen an increase in our understanding of immunology and further expansion in the clinical use of immunotherapeutic modalities. How immunotherapy will be integrated with... Show moreThe past 25 years have seen an increase in our understanding of immunology and further expansion in the clinical use of immunotherapeutic modalities. How immunotherapy will be integrated with chemotherapy, radiation, and surgery remains to be established. Although there have been successes in the field of immunotherapy, they have been inconsistent, and it is hoped that increased understanding of the basic principles of immunology will improve the consistency of beneficial effects. In this article, we briefly provide a general overview of our current understanding of the immune system, with a focus on concepts in tumor immunology, followed by a discussion of how these concepts are being used in the clinic. Show less
Faaij, C.M.; Annels, N.E.; Ruigrok, G.; Burg, M. van der; Ball, L.M.; Bredius, R.G.; ... ; Lankester, A.C. 2010
