Summary:dence or severity of acute GVHD and has the beneficial effect of accelerated engraftment as compared to the use of marrow alone, probably on the basis of an increased num-A 32-year-old male received an allogeneic peripheral blood stem cell transplant (alloPBSCT) for myelodysber of stem cells in the graft. Even though acute GVHD does not appear to be increased with alloPBSCT, we could plasia from his one HLA-A antigen mismatched brother. He is alive with trilineage engraftment and not be certain that the increased T cell content of the PBSC graft compared with marrow, might not effect immediate without active GVHD 200 days after transplant. In July 1986 he underwent orthotopic cardiac transplantation cardiac allograft rejection. Despite the immediate potential benefit over the use of bone marrow alone, there is anecfor viral cardiomyopathy and has received continuous immunosuppressive therapy. A post-transplant lymphodotal evidence for increased extensive chronic graft-versushost disease. The possible correlation with chronic organ proliferative disorder with Hodgkin-like histopathology was diagnosed in August 1993 and was successfully rejection is of concern. Overall, the potential for breaking tolerance for the transplanted solid organ and effecting its treated with four cycles of MOPP chemotherapy. Due to persistent pancytopenia he underwent a bone marrow rejection was of grave concern. We report successful allogeneic peripheral blood stem aspiration and biopsy in May 1996 which revealed monosomy 7 and morphologic changes compatible with cell transplantation for myelodysplasia secondary to chemotherapy for a Hodgkin-like post-transplant lymmyelodysplasia. This is the first report of a cardiac transplant recipient receiving an allogeneic hematopophoma in a recipient of a cardiac transplant. There was no evidence of cardiac graft rejection and engraftment was ietic stem cell transplant. Keywords: allogeneic; cardiac; stem cell; lymphoma; prompt and sustained. myelodysplasia; transplant Case report Myelodysplasia and secondary acute leukemia are wellA 23-year-old male underwent orthotopic cardiac transplandescribed complications of chemotherapy for malignant tation for a viral cardiomyopathy in July 1986. The postdisease.1 Both have been successfully treated with allocardiac transplant course was complicated by mild rejection geneic bone marrow transplantation.2,3 Post-transplant lymas demonstrated on multiple cardiac biopsies, and he phoma is an increasingly common complication among remained on cyclosporin A until FK506 was started prior solid organ transplant recipients and generally has a poor to alloPBSCT. The patient noted increasing adenopathy in outcome. 4 Recently, several reports of successful treatment May 1993 and biopsy of a right axillary lymph node in July with chemotherapy have appeared. 5,6 As the number of 1993 revealed post-transplant lymphoproliferative disease. long-term survivors of solid organ transplantationThe lesion was characterized by a small population of increases, post-trans...
Reduced intensity conditioning regimen with fludarabine, cyclophosphamide, low dose TBI and alemtuzumab leading to successful unrelated umbilical cord stem cell engraftment and survival in two children with dyskeratosis congenita Bone Marrow Transplantation (2016) 51, 744-746; doi:10.1038/ bmt.2015; published online 25 January 2016 Dyskeratosis congenita (DC) is a rare genetic disorder characterized by a classic triad of physical findings consisting of nail dystrophy of the hands and/or feet, mucosal leukoplakia and reticular pigmentation of the skin, most commonly on the head, neck and trunk. 1 Bone marrow failure along with pulmonary complications and malignancies are all common causes of premature death in patients with DC as well as a myriad of other abnormalities. 2,3 Hematopoietic stem cell transplantation (HSCT) with fully myeloablative regimens has historically been the only curative treatment. 2,3 Attempts at reducing morbidity and mortality post HSCT with reduced intensity conditioning regimens instead of myeloablative regimens have been reported, and over the past decade, experience treating DC associated marrow failure with reduced intensity HSCT has increased. 4 Here we report the HSCT course of two patients with successful restoration of hematopoiesis following reduced intensity conditioning and unrelated cord blood transplant (see Table 1 for patient descriptions).Patient 1 Patient 1 is a 9-year-old white male with a history of macrocephaly. He was diagnosed with severe aplastic anemia at the age of 5 years. Approximately 1.5 years before this diagnosis, he had an event where the tip of his tongue became white, jagged and painful. He presented to his primary care physician where it was treated as a viral infection. Concomitantly, circular flat scalp lesions appeared on his head followed by hair loss and crusted patches. He also had a history of easy bruising for 18 months and dysplastic nails. He was hospitalized multiple times for immunosuppressant therapy with cyclosporine and antithymocyte globulin (ATG) over the previous year. A bone marrow biopsy was obtained and showed markedly hypocellular marrow with cellularity o10% on average and tri-lineage hypoplasia with no evidence of malignancy. Telomere length testing was done and shown to be less than the first percentile in all lymphocyte subset and granulocytes, supporting a diagnosis of DC. Genetic testing revealed heterozygosity for R282H mutation in the TINF2 gene. He was referred for allogeneic stem cell transplantation. He did not have a fully matched sibling and an extensive unrelated donor search did not reveal a fully matched unrelated donor nor a good size, well matched umbilical cord. He received 2 partially matched unrelated umbilical cords; 4/6 with a total neutrophil count (TNC) 9.2 x 10 7 and 5/6 with a TNC of 2.2 x 10 7 /kg. The preparative regimen consisted of cyclophosphamide 50 mg/kg x 1, fludarabine 40 mg/m 2 x 5, low dose TBI at 200cGy, and alemtuzumab 0.2 mg/kg x 5. GvHD prophylaxis consisted of tacrolimus and mycophenolate...
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