Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient’s plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered

Case Report Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable. Methods. The patient's plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours. Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity. Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered

Nerve regeneration in vascularized composite allotransplantation: current strategies and future directions

Vascularized composite allotransplantation (VCA) has emerged as a viable treatment option for limb and face reconstruction of severe tissue defects. Functional recovery after VCA requires not only effective immunosuppression, but also consideration of peripheral nerve regeneration to facilitate motor and sensory reinnervation of donor tissue. At the time of transplantation, the donor and recipient nerves are typically coapted in an end-to-end fashion. Following transplantation, there are no therapies available to enhance nerve regeneration and graft reinnervation, and functional outcomes are dependent on the recipients’ innate regenerative capacities. Functional outcomes to date have been promising, but there is still much room for improvement, studies have demonstrated reliable return of protective sensation (pain, thermal, gross tactile), while discriminative sensation and motor function show more inconsistent results. In order to maximize the benefit afforded to the by VCA, we must develop consistent and reliable procedures and therapies to ensure effective nerve regeneration and functional outcomes. New technologies, such as nerve guidance conduits and fibrin glues, and the use of stem cells to facilitate nerve regeneration remain untested in VCA but are proving worthwhile in the context of peripheral nerve repair. VCA presents a unique set of challenges with regards to surgical techniques, postoperative regimen, and health of donor tissue. In this review, we discuss current challenges underlying achievement of nerve regeneration in VCA and discuss novel technologies and approaches to translate nerve regeneration into functional restoration

Malignant catecholamine-secreting carotid body paraganglioma.

The second known case of a malignant catecholamine-secreting (DA)-secreting carotid body paraganglioma is presented. Dopamine synthesis and secretion can be increased in malignant tumors derived from neural crest cells. Whether this is true, in addition, for extra-adrenal paragangliomas is not yet clear. Malignant paragangliomas of the carotid body and larynx, although rare, frequently have been accompanied by increased catecholamine secretion. Malignant catecholamine-secreting carotid body paragangliomas are best treated by composite resection (internal carotid artery and neck dissection), with special attention being given to measures preventing severe hypertension and arrhythmias in the perioperative period

Bioprosthetic Tissue Matrices in Complex Abdominal Wall Reconstruction

Background: Complex abdominal defects are difficult problems encountered by surgeons in multiple specialties. Although current evidence supports the primary repair of these defects with mesh reinforcement, it is unclear which mesh is superior for any given clinical scenario. The purpose of this review was to explore the characteristics of and clinical relevance behind bioprosthetic tissue matrices in an effort to better clarify their role in abdominal wall reconstruction. Methods: We reviewed the peer-reviewed literature on the use of bioprosthetic mesh in human subjects. Basic science articles and large retrospective and prospective reviews were included in author’s analysis. The clinical performance and characteristics of 13 bioprosthetic tissue matrices were evaluated. Results: The majority of the products evaluated perform well in contaminated fields, where the risk of wound-healing difficulties is high. Clinical outcomes, which included infection, reherniation, and bulge formation, were variable, and the majority of the studies had a mean follow-up of less than 24 months. Conclusions: Although bioprosthetic matrix has a multitude of indications within the growing field of abdominal wall reconstruction, the functionality, regenerative capacity, and long-term fate of these products have yet to be fully established. Furthermore, the clinical performance, indications, and contraindications for each type of matrix need to be fully evaluated in long-term outcome studies