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Graft rejection is a common problem after alternative donor transplantation for patients with refractory severe aplastic anemia (SAA). Intensification of the conditioning regimen, with the inclusion of irradiation, has often been advocated to combat this problem. With this approach engraftment rate improved, but the incidence of transplant-related complications is also increased, resulting in little change in the overall outcome. We investigated the use of the combination of fludarabine, cyclophosphamide and anti-thymocyte globulin as the conditioning regimen in five multiply-transfused SAA patients. Three patients received an HLA one-antigen disparate related donor transplant, while two patients were given marrow from matched, unrelated donors. The regimen was well tolerated, with only grade I toxicity encountered. With a median follow-up of 9 months, all patients are alive with complete donor chimerism. We conclude that fludarabine may be used in place of irradiation to augment the conditioning regimen of cyclophosphamide and anti-thymocyte globulin for alternative donor transplantation in children with SAA. Bone Marrow Transplantation (2001) 27, 125–128. [ABSTRACT FROM AUTHOR]

Primary cervical spine osteosarcoma is rare and is considered to have a poor prognosis. We describe an adolescent patient suffering from primary cervical spine osteosarcoma with delayed diagnosis. Nineteen months deficit and symptom-free survival was achieved after an aggressive multi-modality treatment comprising surgery and adjuvant chemoradiotherapy., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Thalassaemia is the most common haemoglobinopathy in the Chinese population. However, recurrent painful digital swelling is not a typical manifestation of this well-known hereditary condition. We describe a case of co-inheritance of beta-thalassaemia and sickle cell trait in a Chinese family and a child who suffered from sickle cell/beta-thalassaemia with recurrent dactylitis. This report highlights awareness of this rare condition in the Chinese population, since acute manifestations can be life-threatening and mimic other emergency conditions. Prompt management can prevent further complications and avoid unnecessary interventions due to delay in diagnosis. A detailed family history and examination of the patient's peripheral blood smear is crucial to reach a correct diagnosis.

Objective: The purpose of this study was to evaluate the role of symptomatology and conventional radiographic scoring in predicting synovial hypertrophy, which could affect the clinical management of hemophilic patients., Materials and Methods: Twenty males (mean age, 14.3 years old) with hemophilic arthropathy, including 34 symptomatic joints and 26 asymptomatic joints (16 knees, 20 ankles, and 24 elbows) had conventional radiographs of individual joints obtained that were rated according to the Arnold-Hilgartner stage and the Pettersson score. The patients also underwent MRI for the detection of synovial hypertrophy. The association of synovial hypertrophy and symptomatology was evaluated using the chi-square or Fisher's exact test. The best sensitivity, specificity, and positive and negative predictive values in detection of synovial hypertrophy using symptomatology and radiographic scoring were calculated., Results: A significant association was seen between symptomatology and synovial hypertrophy of the knee and ankle joints (p < 0.05). The sensitivity, specificity, and positive and negative predictive values of symptomatology in detection of synovial hypertrophy of the knee were 100%, 78%, 78%, and 100%, respectively, and for the ankle were 83%, 75%, 83%, and 75%, respectively. The Arnold-Hilgartner stage and Pettersson score of the radiograph had a significant association with synovial hypertrophy of the knee and ankle joints (p < 0.05). Arnold-Hilgartner staging provided a better prediction of synovial hypertrophy, with sensitivity, specificity, positive predictive value, and negative predictive value of 100%, 100%, 100%, and 100% for knees and 82%, 100%, 100%, and 82% for ankles., Conclusion: In hemophilic patients, the presence of symptomatology in the knee and ankle joints is associated with synovial hypertrophy, and scoring of the conventional radiographs using Arnold-Hilgartner staging is useful for the prediction of synovial hypertrophy.

Background: Pancreatoblastoma is a rare tumour of childhood. Reports of the imaging appearances are limited., Objective: To define the imaging features of pancreatoblastoma by analysis of four previously unreported cases and review of the literature., Materials and Methods: Findings at CT (n = 4), US (n = 3) and MRI (n = 2) were retrospectively reviewed in four patients with pancreatoblastoma. A Medline search was performed to identify relevant literature., Results: Pancreatoblastoma arises most frequently in the body and/or tail, or involves the entire pancreas. Ultrasonography, CT and MRI show variable imaging features, but should in most cases permit preoperative distinction of pancreatoblastoma from other tumours that occur in this region in infancy and childhood. Detection of metastases in the liver, lymph nodes and peritoneal cavity is not significantly better with any one of these three modalities., Conclusion: Preoperative imaging with US, CT and/or MRI will usually suggest a correct diagnosis of pancreatoblastoma. Contrary to previous reports, the tumour arises in the pancreatic head in a minority of cases.