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Introduction Turoctocog alfa (NovoEight® ) is a B-domain-truncated recombinant factor VIII (FVIII) approved for patients with haemophilia A. Aim To investigate the long-term safety and efficacy of turoctocog alfa in routine clinical practice. Methods Guardian 5 was a prospective, multinational, non-interventional, post-authorisation safety study. Male previously treated patients (> 150 exposure days [EDs]) of any age with severe/moderately severe haemophilia A (FVIII ≤ 2%) and a negative inhibitor test prior to first dosing (independent of FVIII-inhibitor history) were included to receive prophylaxis or on-demand treatment. The primary endpoint was the proportion of patients developing FVIII inhibitors (≥.6 Bethesda Units [BU]) after baseline visit, measured as per routine practice of each study site during clinic visits. Secondary endpoints included haemostatic effect, annualised bleeding rate (ABR), and adverse reactions assessment. The study concluded when 50 patients reached 100 EDs/patient minimum. Results Seventy patients were screened and 68 exposed to turoctocog alfa; 63 (92.6%) were on prophylaxis and five received on-demand treatment. Six (8.8%) patients reported a history of positive inhibitors. During the study, patients were exposed to turoctocog alfa for a mean (standard deviation) of 131.9 (99.0) days/patient. Fifty-five of 58 patients who completed the study were tested for FVIII inhibitors; no positive tests were reported. Overall success rate of turoctocog alfa for treatment of bleeds was 87.3%. Among patients receiving prophylaxis, median (range) ABR was 1.97 (.0-25.5) bleeds/year; estimated ABR (negative binomial model) was 3.65 (95% confidence interval: 2.53-5.25). Conclusion Turoctocog alfa was safe and efficacious for haemophilia A treatment in routine clinical practice.

Ewing sarcoma is a rare tumor that requires complex multidisciplinary management. This report describes the general management and standard radiotherapy guidelines in both North America (Children's Oncology Group) and Europe (International Society of Pediatric Oncology). Standard treatment involves multiagent induction chemotherapy followed by local treatment with surgery, definitive radiation, or a combination of surgery and radiation followed by additional chemotherapy and consolidation local treatment to metastatic sites. The data supporting the role of chemotherapy, surgery, and radiation and specific radiation therapy guidelines are presented.

BACKGROUND It has been suggested that hemoglobin levels of 12–14 g/dL are optimal for tumor oxygenation, radiosensitivity, and prognosis. In this prospective study, the authors evaluated the effectiveness of epoetin-α to maintain hemoglobin levels at 12–14 g/dL during radiotherapy (RT) for patients with UICC Stage III esophageal carcinoma, and they examined the impact of erythropoetin on overall survival (OS), metastatic-free survival (MFS), and local control (LC). METHODS Sixty patients who received RT between March, 2001 and September, 2004, were included in this prospective, nonrandomized study. Thirty patients received epoetin-α (150 IU/kg 3 times per week) during RT (Group A), and 30 patients did not receive epoetin-α (Group B). Epoetin-α was started at hemoglobin levels < 13 g/dL and was stopped at hemoglobin levels ≥ 14 g/dL. Hemoglobin was measured before RT and once weekly during RT. RESULTS Both patient groups were balanced for age, gender, performance status, tumor location/length, histology, grading, tumor classification, lymph node status, chemotherapy, treatment (45–50.4 grays [Gy] plus resection vs. 45.0–50.4 Gy vs. 59.4–66.0 Gy), and hemoglobin level before RT. In 20 of 30 patients (67%) from Group A and in 3 of 30 patients (10%) from Group B, ≥ 60% of hemoglobin levels during RT were 12–14 g/dL (P = 0.003). The median change in hemoglobin was + 0.4 g/dL per week in Group A and − 0.4 g/dL per week in Group B. LC was significantly better in Group A (66% vs. 38% at 1 year, respectively; P = 0.012), a trend was observed for OS (59% vs. 33%, respectively; P = 0.08), and MFS did not differ significantly (43% vs. 38%, respectively; P = 0.34). No epoetin-α-related toxicity was observed. CONCLUSIONS Epoetin-α was effective in maintaining the hemoglobin levels at 12–14 g/dL during RT. The application of epoetin-α significantly improved LC, and a trend was observed for OS. Cancer 2005. © 2005 American Cancer Society.

We report on two girls, 3 and 13 years old, with acute promyelocytic leukemia (APL) who were successfully treated with all-trans retinoic acid (ATRA) 45 mg/m 2 /day. Retinoic acid syndrome was prevented with short-time treatment of high dose (4 x 1.5 g/m 2 ) cytarabine. This regimen was well tolerated, although both children were critically ill. They achieved a complete remission confirmed by light microscopy, but reverse transcriptase polymerase chain reaction remained positive after ATRA, underlining the need of further chemotherapy.

Durch Umsetzen von Thionylchlorid mit Ammoniak in der Warme bei Anwesenheit eines organischen Losungsmittels kann man festes rotviolettes HOSN erhalten. Mit Thionylchlorid im Uberschus entsteht daraus Tri-thiazylchlorid, (NSCl)3; durch Umsetzen mit NH3 wird Imidodisulfinamid gebildet. Durch Reaktion mit Triphenyl-methylnatrium konnte Na[OSN] hergestellt werden, das viel stabiler ist als die Wasserstoffverbindung.