1/40. The management of a person with haemophilia who has a fixed flexed hip and intractable pain.The clinical picture of a fixed flexed hip associated with pain in a person with haemophilia is suggestive of a haemorrhage in that area. Sonography facilitates differentiation between a haemarthrosis, intraperitoneal haemorrhage, subperiosteal bleed, a bleed into the soft tissue around the hip joint or a psoas haematoma. All these aforementioned causes may result in the same clinical presentation. Two cases are described in which coxhaemarthrosis resulted in a flexion contracture of the joint associated with severe intractable pain. Narcotic drugs failed to alleviate the severe pain. Joint aspiration produced dramatic pain relief and early joint rehabilitation.- - - - - - - - - - ranking = 1keywords = haematoma (Clic here for more details about this article) |
2/40. Spinal epidural haematoma in haemophilia A with inhibitors--efficacy of recombinant factor viia concentrate.We report the case of a 21-year-old man with severe haemophilia A and factor viii inhibitors who presented with an extensive spinal epidural haematoma (C2-T12), probably induced by sit-up exercises. The bleed was defined by magnetic resonance imaging of the cervical and thoracic spine and prompt treatment with recombinant factor viia concentrate led to complete resolution at 4 weeks. Neurological sequelae were averted and surgical decompression was not necessary. We discuss the difficulties in diagnosis and management of such a case.- - - - - - - - - - ranking = 5keywords = haematoma (Clic here for more details about this article) |
3/40. Expanding portal haematomata as a complication of knee arthroscopies in persons with haemophilia.Recurrent haemarthroses stimulate the hypertrophy of synovial tissues that if left in situ will eventually cause joint destruction. Synovectomies have been the cornerstone of joint preservation and a number of different methods exist. We report two patients who suffered complications after an arthroscopic procedure. No previous complications of this nature have been reported in the literature.- - - - - - - - - - ranking = 4keywords = haematoma (Clic here for more details about this article) |
4/40. Major surgery for a gastric cancer in a haemophilic with high inhibitor titre successfully performed by the use of recombinant FVIIa.A total gastrectomy with omentectomy and resection of the distal oesophagus in a 69-year-old haemophilia A patient with high inhibitor of 128 Bethesda units is described. Surgery was successfully performed after infusion of 112 microg kg-1 bw of recombinant FVIIa. Ninety-two microg kg-1 were given thereafter at time intervals of 2 h until 12 h, then every 3 h until 24 h, and every 4 h until 48 h after surgery. Doses were gradually reduced in the following days and finally discontinued on day 28 after surgery. The complete treatment schedule required the administration of a total of 708 mg of recombinant FVIIa. Using this approach, we observed normal haemostasis, and there were no signs of excessive postoperative bleeding or wound haematoma. No clinical side-effects or evidence of systemic activation of coagulation occurred during the treatment. As judged from the clinical course of this major surgery, recombinant FVIIa appears to be highly efficacious and safe and should be used as first line treatment in high titre inhibitor patients with cross-reactivity to porcine factor viii, undergoing surgery.- - - - - - - - - - ranking = 1keywords = haematoma (Clic here for more details about this article) |
5/40. Thrombosis in a duplicated superficial femoral vein in a patient with haemophilia A.venous thrombosis is a very rare occurrence in patients with haemophilia A. We report the case of a haemophiliac in whom initially a calf haematoma was suspected, but neither this nor deep venous thrombosis (DVT) could be confirmed on ultrasound scanning. Subsequently, a high segment venous thrombosis was diagnosed by venography in a portion of a duplicated superficial femoral vein. Treatment with factor viii (FVIII) and low molecular weight heparin led to a successful resolution. The only other case we have been able to find in the literature occurred during FVIII replacement therapy, which was not the situation with our patient.- - - - - - - - - - ranking = 1keywords = haematoma (Clic here for more details about this article) |
6/40. portal vein thrombosis in a patient with severe haemophilia A and F V G1691A mutation during continuous infusion of F VIII after intramural jejunal bleeding--successful thrombolysis under heparin therapy.We report on a 14-year-old boy with severe haemophilia A who developed a portal vein thrombosis during continuous infusion of F VIII. For treatment of a posttraumatic intramural jejunal haematoma with extension into the mesenterium the patient received continuous infusion (CI) of a high purity F VIII concentrate, starting with an initial bolus injection of 100 IU F VIII/kg bw and followed by 4-5 IU F VIII/kg bw/h i.v. F VIII plasma activity ranged between 47 and 88%. Resorption of the haematoma was proven by abdominal ultrasonic follow-ups. After 3 weeks of CI a thrombus formation in the portal vein was detected by ultrasound and confirmed by duplex ultrasound. Subsequent to diagnosis the patient was heparinised with unfractionated heparin (UFH 300-450 IU/kg/d i.v.). In order to induce further resorption of the haematoma. F VIII concentrate was given concomitantly (50 IU/kg bw twice daily) during the initial phase of treatment. After 14 days of anticoagulant therapy with UFH, the regimen was changed to low molecular weight heparin (LMWH; Fraxiparin 0.3; 2850 IU anti-X activity/d s.c.; bw 60 kg). F VIII dosage was gradually reduced with advanced resorption of the haematoma and thereafter switched to prophylaxis (40 IU/kg bw 3 times weekly). Complete lysis of the thrombus was observed after 6 months of treatment with UFH and LMWH respectively without any further complications. Thereafter LMWH was discontinued. Thrombophilic screening revealed no abnormalities except heterozygous F V G1691A. CONCLUSION: The coexistence of a common prothrombotic risk factor and haemophilia may cause severe complications, in particular if the bleeding disorder has to be corrected temporarily by administration of the concerning deficient agent.- - - - - - - - - - ranking = 4keywords = haematoma (Clic here for more details about this article) |
7/40. Drug-induced and traumatic nail problems in the haemophilias.Many persons with haemophilia suffer from hiv and receive highly active antiretroviral therapy. Three patients received indinavir and required surgery due to ingrown toenails. Two patients suffered from a traumatic subungual haematoma. The treatment protocol is described whereby the pressure exerted onto the germinal layer and the nail bed is relieved in order to alleviate pain and nail matrix damage.- - - - - - - - - - ranking = 1keywords = haematoma (Clic here for more details about this article) |
8/40. Experience with continuous infusion of recombinant activated factor VII in the asia-Pacific region.There is increasing interest in continuous infusion of recombinant activated factor VII (rFVIIa) as a convenient and safe alternative to intermittent bolus therapy. In the Australian patients reported in this paper, cost savings of up to 25% in the first 12 h of treatment with continuous infusion of rFVIIa have been achieved safely, suggesting that substantial overall savings are possible. However, in the Thai patient reported, a dose reduction of 35% in the first 12 h was associated with poor haemostatic control, suggesting that a dose reduction of >25% may be inadvisable. The indications for treatment in the five Australian patients were: retroperitoneal haemorrhage (n = 3); right forearm compartment syndrome (n = 1); wrist haemarthrosis and median nerve compression (n = 2); sublingual haematoma (n = 1); and cerebral (mid-brain) haemorrhage (n = 1). Treatment was effective in four out of five patients (six bleeding episodes) and there was one treatment failure where treatment had been substantially delayed. The Thai patient was treated as part of a prospective, uncontrolled, observational study of 34 bleeding episodes in 22 patients in the asia-Pacific region. Treatment was judged ineffective after 24 h, but full haemostatic control was subsequently achieved with intermittent rFVIIa therapy.- - - - - - - - - - ranking = 1keywords = haematoma (Clic here for more details about this article) |
9/40. Surreptitious bleeding in surgery: a major challenge in coagulation.Apart from inadequate surgical haemostasis, postoperative bleeding can be related to acquired disorders of platelet number, platelet function or coagulation proteins (e.g. vitamin k deficiency, DIC or liver injury). We highlight our experience with three patients who suffered life-threatening bleeding in the postoperative setting. The three patients - a 47-year-old man and 70- and 74-year-old women -- all had negative histories for excessive bleeding with prior surgeries, and all had normal preoperative PT and aPTT tests. Surgeries were resection of ischaemic bowel, cholecystectomy and coronary artery bypass grafting. All patients experienced unexpected bleeding within the first few postoperative days requiring multiple red cell transfusions and surgical re-explorations. Evaluations within the first 4--7 days after surgery revealed that these three patients had developed prolonged aPTT due to demonstrable factor viii antibodies initially at low titre. One patient was treated with high doses human factor viii, corticosteroids, intravenous gammaglobulin and plasma exchanges. The inhibitor was no longer demonstrable after 6 weeks of such therapy, and he has remained in remission without therapy. The second patient was initially treated with high-dose human factor viii infusions. Five months later, prednisone and 6-mercaptopurine were begun for worsening inhibitor titre and diffuse purpura and subcutaneous haematomas. The factor inhibitor remitted, but the patient died from liver failure related to post-transfusion hepatitis. The third patient was initially managed with high-dose human factor viii. Two months later, worsening inhibitor titre and tongue haematoma was treated with activated prothrombin complex, corticosteroids and cyclophosphamide. Eight years later, she is on no therapy, demonstrates a mild bleeding tendency and has a stable low-titre inhibitor. There have been a few case reports of inhibitors to coagulation factors including factor viii becoming manifest in the postoperative setting but surgery has not been widely recognized as an underlying cause for acquired haemophilia. This paper speculates on pathogenesis and reviews treatment options. This syndrome is remarkable for its abrupt onset in the first few postoperative days and for its substantial morbidity. The problem is potentially reversible with immunosuppressive therapy. Clinicians should be aware of this syndrome, considering acquired haemophilia in patients with unexpected postoperative bleeding.- - - - - - - - - - ranking = 2keywords = haematoma (Clic here for more details about this article) |
10/40. Blunt abdominal trauma with delayed rupture of splenic haematoma in a haemophiliac patient.A 13-year-old haemophilia A patient presented with pain in the abdomen, 4 days after a blunt abdominal trauma. The computed tomography scan of the abdomen showed a large splenic haematoma. The patient was initially managed with factor viii replacement therapy, but 4 weeks later he had a delayed rupture of the splenic haematoma with haemoperitoneum and shock. An elective splenectomy under factor viii therapy was successfully performed.- - - - - - - - - - ranking = 6keywords = haematoma (Clic here for more details about this article) |
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