The clinical features of acute portal vein thrombosis (APVT) are poorly defined in the literature. The proportion that progress to chronic PVT and the influences of various treatments are unknown. Between 1996 and 1998, nine patients presented to our hospital with varying upper gastrointestinal symptoms. They were found to have APVT by colour flow Doppler ultrasound, which was confirmed by CT scanning. All were tested for procoagulant tendencies and then treated with intravenous heparin for 7 days and warfarin for 3 months. Colour flow Doppler ultrasound or CT was done regularly to assess response to treatment. There was complete resolution of thrombus in five patients. Four patients had procoagulant tendencies identified; of these the thrombus resolved in two cases and in two cavernous transformation occurred. In most cases, the thrombus disperses on heparin and warfarin, although the effect of this therapy is unknown. A randomized trial of thrombolytic therapy may be appropriate, in an attempt to reduce the rate of progression to chronic PVT.
Autologous (ASCT) and allogeneic stem cell transplantations (alloBMT) are well-established therapies for multiple myeloma. However, patients continue to relapse at a constant rate. We present here 15 out of 163 patients who underwent SCT and relapsed with plasmacytomas only without evidence of bone marrow disease progression (14/147 post-ASCT and 1/16 post-alloBMT). The median time from SCT to plasmacytoma relapse was 24 months. The sites of plasmacytoma included bone, skin, rectum, and testicles. Five patients were treated with local radiotherapy, while seven patients received a combination of radiotherapy and chemotherapy or thalidomide, and two patients received chemotherapy alone with or without thalidomide. The recipient of alloBMT was initially treated with VAD-chemotherapy and local radiotherapy followed by a mini-allograft from the original donor. Eleven patients died at a median of 10 months following diagnosis of the plasmacytoma. Four are still alive, 12-20 months post-plasmacytoma diagnosis. These cases of unconventional disease recurrence are likely to be seen due to sub-clinical seeding of tumour cells suggestive of the presence of an extramedullary (EM) clone of plasma cells with a high degree of chemoresistance. We also review all the available data in the literature for the optimal therapy for patients with isolated EM relapse.
The contribution of losses via the gastrointestinal tract to maintenance amino acid requirements was assessed by collecting the ileostomy fluid of volunteers given a proteinfree diet for 5 d. The subjects were eight adult men and women with terminal ileum ileostomies after ulcerative colitis. Four consecutive 24-h collections of both digesta and urine were made. On the last 2 d an antibiotic was given that suppressed microbial activity in the digesta and slightly reduced ileostomy outflow. Mean daily amino acid excretion in ileostomy fluid ranged from 32 mg/d for methionine to 330 mg/d for aspartate and glutamate. These losses were compared with current international estimates of amino acid requirements. For most essential amino acids gastrointestinal losses accounted for 14-33% of daily maintenance requirements but for threonine the contribution was 61%.
This systematic review found low-quality evidence of an association between PF and weight-bearing tasks such as walking and standing on hard surfaces. The only occupations specifically identified as having higher risk were those associated with the engine assembly plant. Further research is required to fully determine the association between weight bearing and PF.
Essential Thrombocythcythaemia (ET) is an uncommon type of myeloproliferative disorder, characterised by both thrombotic and haemorrhagic diathesis. No clear guidelines exist for the preand post-operative management of patients undergoing cardiac surgery in the haematological and surgical literature. This condition has profound implications in patients undergoing cardiac surgery with the use of cardiopulmonary bypass, where heparin is used for anti-coagulation. This dilemma is further compounded in the setting of a young patient undergoing aortic valve replacement (AVR), where insertion of a mechanical prosthesis would be the procedure of choice. This would require life-long anticoagulation with warfarin which can predispose these patients to catastrophic bleeding. Using a tissue valve will subject the patient to multiple redo operations in the patient's lifetime. We report a young patient with ET requiring AVR and discuss the dilemmas surrounding the choice of prosthesis in this patient. Case reportA 22 year old gentleman was referred to our cardiothoracic centre for consideration for aortic valve replacement (AVR). He was diagnosed with moderate aortic valve stenosis at birth. The aortic valve was bicuspid and there was no associated significant left ventricular hypertrophy as a neonate. He had no other past significant medical history except for migraine. He was asymptomatic. At the age of 5 years the peak gradient (PG) across the aortic valve was 30 mmHg on trans-thoracic echocardiogram (TTE). At follow-up, 1 year before referral, there was an increase in the PG to 50 mm Hg followed by an increase to 86 mm Hg two weeks before referral. The patient denied any symptoms. At the age of 21, he was found to have an isolated thromocytosis with a platelet count of 874 × 10 9 /L with a normal haemoglobin, haematocrit and white blood cell count. His inflammatory markers were negative. The platelet count was repeatedly above 800 × 10 9 /L. There was no history of bleeding or thrombocytosis and no splenomegaly on examination. He was referred to a haematologist. A bone marrow aspirate showed increased number of megakaryocytes. The bone marrow trephine showed classical Essential Thrombocythaemia (ET) (figure 1). Cytogenetics were normal. Blood was negative for JAK-2 gene. In view of his age (<40 yrs), absence of hypertension and diabetes, he was categorised as low risk and started on aspirin alone. However, the planned aortic valve surgery necessitated controlling the platelet count over the perioperative period to reduce his risk of bleeding or thrombosis. The options were short-term use of hydroxycarbamide with a theoretical leukaemia and teratogenic risk or α-interferon. The patient elected to have α-
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