Wiener klinische Wochenschrift
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Wien. Klin. Wochenschr. · Jun 2022
Successful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy : Breaking a dangerous bond.
Acquired von Willebrand syndrome is exceedingly rare and accounts for only 1-3% of von Willebrand disease cases. In this short report, we present our own cases of acquired von Willebrand syndrome associated with monoclonal gammopathy. Both cases went into complete and sustained remission after intensive antimyeloma treatment. ⋯ After at least VGPR was achieved the coagulation studies rapidly normalized and remained normal after treatment de-escalation to lenalidomide/dexamethasone maintenance. The second patient successfully underwent ASCT after 5 cycles of induction with daratumumab, bortezomib, cyclophosphamide and dexamethasone and has remained in full hematologic and hemostaseologic remission ever since. The two cases highlight the efficacy of aggressive antimyeloma treatment in monoclonal gammopathy-associated acquired von Willebrand syndrome to achieve normalization of coagulation study, providing a possible way to manage these patients.
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Tuberculosis is among the leading causes of death from infectious diseases and affects many organ systems, including the skeleton. Skeletal tuberculosis is an extrapulmonary stage of tuberculosis, which occurs after the early and post-primary pulmonary stages of the disease. The aim of our study was to assess the microarchitecture of historic dry bone samples of subjects who have died of tuberculosis documented by post-mortem examinations. ⋯ Cortical porosity was seen in affected vertebrae and tibia; in tuberculous tibiae (but not in the femora) cortical thickness was decreased. In half of the individuals, cortical sclerosis was present; signs of ankylosis were observed mainly at the femoral heads affected with tuberculosis. We conclude that a combination of several alterations at the trabecular compartment could be suggestive of the presence of tuberculosis in historic skeletal remains.
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Wien. Klin. Wochenschr. · Jun 2022
Evaluation of the effect of preoperative hemoglobin level and proinflammatory factors on intertrochanteric fracture union.
Intertrochanteric fractures are associated with high mortality and morbidity, so these patients should undergo fracture fixation surgery immediately. Despite surgery, the possibility of fracture fusion may not occur due to the association with various causes. Therefore, our aim is to investigate these factors (TNF‑a, IL‑1, Hb) and their effect on fracture union after fixation. ⋯ Due to the effect of anemia and proinflammatory factors in the process of healing fractures and bone formation and creating musculoskeletal balance, low hemoglobin level before surgery has a significant effect on fracture union and failure of fixation. So it is recommended to correct this anemia in these patients before surgery and during follow-up.
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Wien. Klin. Wochenschr. · Jun 2022
Long-term eliglustat treatment of Gaucher patients over up to 10 years in Vienna.
Gaucher disease has been the first lysosomal storage disorder for which an enzyme replacement therapy has been approved in the 1990s and was the first to receive approval for a first-line substrate reduction therapy in 2015. Eliglustat treatment has been started in Austria in patients recruited to a clinical trial, followed by its long-term extension and prescription treatment overall covering up to 10 years. In this case series the experience of treating Gaucher patients with eliglustat in Vienna is summarized. ⋯ Stability was found in switch patients with slight improvement in bone density. Eliglustat was generally very well tolerated. Patient selection and regular monitoring is required to ensure effective and safe use.