Summary:Children with Wilms tumor who have a particular risk of failure at relapse or at primary diagnosis were treated with high-dose chemotherapy (HDC) and autologous peripheral blood stem cell rescue in order to improve their probability of survival. Keywords: Wilms tumor; high-dose chemotherapy; autologous bone marrow transplantation A child with newly diagnosed Wilms tumor (WT) has a probability of about 85% of being cured with multimodal treatment nowadays. Current treatment strategies stratify intensity and scheduling of the different treatment modalities -surgery, radiation and chemotherapy -according to biologic and prognostic features of the initial disease, most importantly, stage and histology. Three large multiinstitutional study groups have contributed to this development: the National Wilms Tumor Study (NWTS), USA, 1-3 the United Kingdom Children's Cancer Study Group 4,5 and the International Society of Pediatric Oncology (SIOP). [6][7][8][9] For the 15% of patients with relapse or refractory disease, attempts have been made to use a similar risk-adapted strategy. These efforts were based upon the analysis of Grundy et al 10 demonstrating a 3-year survival of 30% in 367 patients who relapsed following treatment within studies NWTS-2 and -3. The authors identified prognostic factors with respect to time and site of relapse, histology and previous therapy, and used them to identify a good-risk group of relapsed patients who had a survival of >40% with conventional therapy. 10 For the complementary group of very high-risk patients, treatment alternatives were investigated. In phase II studies, single drugs or combinations gave the following results: single drug etoposide: 42% of response; 11 ifosfamide plus etoposide: overall response rate 39.5%; 12 etoposide and ifosfamide: 15% complete and 54% partial remission; 13 etoposide plus carboplatin: 70% response. 14 None of these approaches, however, could improve the long-term survival of these patients above 30%. 14 Consequently, dose-response strategies were investigated. Warkentin et al 15 who administered etoposide and thiotepa, reported eight out of 12 relapse-free survivors after 1 year. The Solid Tumor Registry of the EBMT reviewed 24
Summary:We report a patient who developed Epstein-Barr virus (EBV)-induced transverse myelitis 19 months after unrelated bone marrow transplantation (BMT). The disease was diagnosed by physical examination, serologic determinations, EBV-specific polymerase chain reaction in peripheral blood lymphocytes and cerebrospinal fluid, and characteristic magnetic resonance imaging scan of the spine. The patient was treated with ganciclovir and cytomegalovirus (CMV) hyperimmune globulin. He gradually improved and recovered completely within 4 weeks. This case suggests that ganciclovir and CMV hyperimmune globulin appear to be effective for the treatment of EBV-induced transverse myelitis in immunocompromised patients following BMT. Keywords: Epstein-Barr virus; transverse myelitis; bone marrow transplantation Epstein-Barr virus (EBV)-associated lymphoproliferative disorder is a well-recognized infectious complication in patients receiving bone marrow transplantation (BMT), particularly from unrelated donors. 1 Other EBV-induced clinical manifestations are relatively infrequent following BMT. Transverse myelitis, an inflammatory disease of the spinal cord, is one of the rarest presentations of EBV infection. 2 We report a patient who developed acute EBVinduced transverse myelitis 19 months following unrelated BMT. The patient recovered completely with ganciclovir and cytomegalovirus (CMV) hyperimmune globulin treatment. Case reportA 16-year-old male received an allogeneic BMT for acute myeloid leukemia (FAB classification M2) in second partial remission after conditioning with busulphan (4 mg/kg/day ϫ 4 days) and cyclophosphamide (50 mg/kg/day ϫ 4 days). The HLA-matched unrelated donor was seronegative for CMV but had reactivation of EBV at the time of transplantation. Before harvest, the donor's EBV serostatus was positive for viral capsid antigen (VCA) immunoglobulin G (IgG), VCA IgM, early antigen (EA) IgG, and EpsteinBarr nuclear antigen (EBNA) antibodies. The recipient was VCA-IgG positive, VCA-IgM negative and EBNA-antibodies positive. Polymerase chain reaction (PCR) for EBV DNA from buffy coat leukocytes was positive in the donor and negative in the recipient. Acyclovir and CMV hyperimmune globulin (Cytotect; Biotest, Dreieich, Germany) with high titers of EBV antibodies were given as prophylaxis for EBV disease in the early post-transplantation period. Graft-versus-host disease (GVHD) prophylaxis consisted of antithymocyte globulin, cyclosporin A, methotrexate, and prednisolone. The patient developed moderate acute GVHD of the skin and the gastrointestinal tract and received methylprednisolone and FK506 (tacrolimus) instead of cyclosporin A. Because of extensive chronic GVHD with generalized skin and eye involvement he was treated with prednisolone and azathioprine.Nineteen months after BMT, the patient presented with local back pain and bilateral lower extremity sensory loss. There was a clear sensory level at the 10th thoracic dermatome, below which pain, temperature, light touch, position, and vibratory sensatio...
Infections caused by nontuberculous mycobacteria (NTM) commonly manifest as lymphadenitis in otherwise healthy children, as pneumonia in patients with chronic airway diseases, and as disseminated disease in patients with immunosuppression. The aim of this case report is to describe imaging findings (sonography, electrical impedance scanning, MRI) in children with NTM infection of the head and neck and to give some information about microbiologic findings and therapeutic options. In children presenting with enlarged neck masses and radiologically demonstrated nodal masses, the diagnosis of infections with NTM should be considered.
The imaging findings requiring immediate treatment even though the children did not show any clinical signs, justify cerebral MR examinations prior to stem cell transplantation.
No standard treatment is available for relapse of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) after allogeneic stem cell transplantation (alloSCT). Efficacy of donor lymphocyte transfusion (DLT) was limited, at least in part, by the rapid pace of the disease, overwhelming any allogeneic immune response. However, induction chemotherapy before DLT was complicated by severe toxicity. Based on a pilot trial conducted in Munich, a prospective, multicenter phase II trial for relapsed AML and MDS after alloSCT was initiated in 2000 by the German transplant cooperative group. The study evaluated the sequence of low-dose(ld)AraC for leukemic control (intensive chemotherapy for progressive disease only), transfusion of donor PBSC without immunosuppression as adoptive immunotherapy, and systemic application of GM-CSF. GM-CSF was included, since in combination with other cytokines, it has been able to improve the antigene presentation capacity of myeloid blasts in vitro. Between 2000 and 2006, 41 patients with hematological relapse of AML or MDS >3 months after alloSCT were included. Median age was 47y, 50% had an unrelated donor. Median remission after SCT was 223d (93–1614), median percentage of BM blasts at relapse was 40%. Median follow up was 28 months. Control of leukemic proliferation by ldAraC was achieved in 61%, allowing outpatient care in the majority of these patients. 39% required intensive chemotherapy. Three patients died from infections before donor cell transfusion (DCT), one patient was not transfused due to progressive leukemia. Median time from relapse to DCT was 52 days. 25/34 evaluable patients were found to be free of blasts in BM at d35 after PBSC and were considered initial responders. In an intent-to-treat analysis, overall survival (OS) of the entire cohort at 1 and 2 years from relapse was 41% and 32%. Among initial responders, 1y- and 2y-OS was 68% and 49%. A remission >6months after alloSCT, and control of leukemia by ldAraC prior to DCT were associated with better outcome. At last follow up, 8 patiens were in continous CR, 2 were alive with second relapse, 18 had died from leukemia, and 13 had died in remission or aplasia. In conclusion, ldAraC seems to be effective for initial control of leukemic proliferation in relapsed AML and MDS after alloSCT. A longer duration of post-transplant remission and response to ldAraC may identify patients who will benefit from adoptive immunotherapy. On an intend-to-treat-basis, the overall results of our trial compare favorably to other published strategies; nevertheless, outcome is still unsatisfying and warrants further investigation.
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