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(1/8) Bone marrow transplantation from matched siblings in patients with fanconi anemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation and antithymocyte globulin.

Nineteen patients with Fanconi anemia (FA) and bone marrow failure underwent bone marrow transplantation (BMT) from matched siblings. Median age at BMT was 8.7 years. Conditioning consisted of low-dose cyclophosphamide (CY 5 mg/kg x 4 days) and thoracoabdominal irradiation (TAI 400 cGy). Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A (CsA) in 13 patients and CsA plus methotrexate in 6 patients. Antithymocyte globulin (ATG) was added in the pretransplant as well as the post-transplant period. All patients received high-dose acyclovir from day 2 pre-BMT to day 28 post BMT, and intravenous immunoglobulins (IVIG), 500 mg/kg weekly from day 7 pre-BMT to day 90 post BMT. No fungal prophylaxis was given. All patients engrafted, (median, 14 days for an absolute neutrophil count > or =0.5 x 10(9)/l; median, 37 days for platelet count > or =20 x 10(9)/l). Fourteen (74%) patients are alive with sustained engraftment and are transfusion independent. Three (16.6%) patients developed acute GVHD; none developed chronic GVHD. Five (26%) patients developed invasive fungal infections, and two (10%) developed fatal CMV disease. We believe the addition of ATG may have contributed to the increased incidence of severe life-threatening fungal and viral infections in our series.  (+info)

(2/8) Needle-track metastases and prophylactic radiotherapy for mesothelioma.

BACKGROUND: Mesothelioma invades the tracts made by chest instrumentation. Prophylactic radiotherapy is effective at preventing malignant seeding at these sites. METHODS: We assessed the use and effectiveness of radiotherapy at our centre in 39 of the 40 patients identified with mesothelioma between January 2000 and September 2003. RESULTS: Thirty-seven (95%) patients received radiotherapy to their chest instrumentation site between 6 and 42 days (median 26 days) following the diagnosis of mesothelioma. The radiotherapy field size varied, from 4 cm square to 14 x 10.5 cm. The radiotherapy was given as 21 Gy in 3 fractions over 1 week. In 3 patients (8%), there was already tumour invasion of the skin at the time of radiotherapy. In 2 other patients (5%), there was tumour recurrence following radiotherapy; in both this was at the edge of the previous radiotherapy fields. Further treatment was administered to an adjacent field in both. One patient with an indwelling pleural catheter developed tumour growth at the catheter insertion site. This was treated successfully with radiotherapy, with no catheter damage. CONCLUSIONS: Prompt radiotherapy referral and radiotherapy field selection is important to maximise the effect of radiotherapy given to prevent chest wall tumour growth. There was no tumour growth in areas that were treated with radiotherapy. Further chest interventions outside the radiotherapy field should be followed with further radiotherapy.  (+info)

(3/8) A phase I trial of tocoferol monoglucoside in patients undergoing hemi-body radiation.

PURPOSE: To evaluate Tocoferol monoglucoside (TMG), a water soluble vit. E. in a phase I trial, as a radiation protector in those undergoing hemi-body radiation for disseminated disease. MATERIALS AND METHODS: Patients scheduled to receive modified hemi-body radiation were accrued for the study. Patients not only had disseminated skeletal disease but, were heavily pretreated Seven patients were accrued for the study. Patients received 1 and 2 gms of TMG. 30-40 minutes before hemibody radiation. A dose of 600 cGy was delivered on telecobalt equipment at mid plane. Immediate Toxicities were evaluated as well as response to pain. RESULTS: All the seven patients underwent radiation uneventfully. There was no drug related toxicity. Pain relief was adequate. CONCLUSION: Tocoferol monoglucoside an effective antioxidant with no significant acute toxicity, when administered in a dose of 1 or 2 gms per oral route. TMG being water-soluble can have global antioxidant and radio protective effects. This needs further clinical evaluation.  (+info)

(4/8) Prevention and treatment of vitamin D deficiency in Dutch psychogeriatric nursing home residents by weekly half-body UVB exposure after showering: a pilot study.

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(5/8) Partial depletion of regulatory T cells does not influence the inflammation caused by high dose hemi-body irradiation.

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(6/8) Synergistic effect of cisplatin and synchrotron irradiation on F98 gliomas growing in nude mice.

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(7/8) Bone marrow transplantation for Fanconi anemia using low-dose cyclophosphamide/thoracoabdominal irradiation as conditioning regimen: chimerism study by the polymerase chain reaction.

Since 1976, patients grafted at the Hopital Saint-Louis for Fanconi anemia (FA) without evidence of leukemic transformation have been given a uniform conditioning regimen that consisted of low-dose cyclophosphamide (Cy) and thoracoabdominal irradiation (TAI). The use of low-dose Cy raised the question of whether it is sufficient for the establishment of a complete hematopoietic chimerism in all patients. We therefore initiated a study of chimerism early during hematopoietic reconstitution after bone marrow transplantation (BMT) and thereafter in transplanted FA patients. Minisatellite probes were used after DNA amplification by the polymerase chain reaction (PCR). From July 1989 to October 1992, 24 consecutive patients underwent BMT for FA, 19 of whom were assessable for chimerism. Our results using this sensitive technique showed that, among these 19 patients, all but one successfully engrafted. Engraftment was complete early after BMT in 12. The persistence of a small proportion of recipient's cells was detected in six. This partial hematopoietic chimerism was demonstrated to be only transient in at least five of the six patients. The one patient who failed to engraft showed a recipient-type profile for circulating cells early posttransplantation, indicating autologous bone marrow recovery. A second graft in this patient was also rejected. For both transplantations, the patient was grafted from a matched, unrelated donor. Therefore, 17 of 17 patients successfully grafted and with complete follow up data presented complete hematopoietic chimerism, within the sensitivity limit of the method used. In conclusion, lowering the Cy dose in the conditioning regimen of patients with FA could still allow complete engraftment to occur, at least in patients with an identical sibling donor.  (+info)

(8/8) Unrelated donor bone marrow transplantation in Fanconi anaemia: the Leiden experience.

Fanconi anaemia (FA) is an accepted indication for treatment with allogeneic HLA-identical BMT. Most patients, however, lack a suitable HLA-identical donor. In our centre, six FA patients were transplanted with a matched unrelated donor. Due to hypersensitivity to DNA cross-linking agents, a low-dose cyclophosphamide (CY) and thoraco-abdominal irradiation (TAI) regimen is recommended for conditioning in FA. We added Ara-C upfront and anti-T cell antibodies to enhance engraftment and to prevent GVHD, in combination with T cell depletion in four out of six of the first transplants. One patient did not engraft. In three patients rejection was observed. In three of these four patients a second BMT, using full bone marrow grafts, resulted in successful engraftment. The other patient died before a second BMT could be performed. The incidence and severity of acute GVHD was low: only one patient with grade III acute GVHD was seen. Two out of four surviving patients suffered from chronic GVHD. Four patients survived (median survival time 43 months after BMT), three with good and one with acceptable quality of life. Two patients died, one patient due to adenoviral reactivation with multi-organ failure, and one due to sepsis complicated by ARDS. In conclusion, MUD BMT is feasible in FA patients with bone marrow failure in whom no HLA-identical sibling donor is available. In our study group, the major problem was graft rejection, despite the administration of a combination of graft enhancing anti-T cell antibodies. Multicentre studies are needed to determine a more intensive, but still tolerable, conditioning regimen.  (+info)