1/517. Systemic infection with Alaria americana (trematoda).Alaria americana is a trematode, the adult of which is found in mammalian carnivores. The first case of disseminated human infection by the mesocercarial stage of this worm occurred in a 24-year-old man. The infection possibly was acquired by the eating of inadequately cooked frogs, which are intermediate hosts of the worm. The diagnosis was made during life by lung biopsy and confirmed at autopsy. The mesocercariae were present in the stomach wall, lymph nodes, liver, myocardium, pancreas and surrounding adipose tissue, spleen, kidney, lungs, brain and spinal cord. There was no host reaction to the parasites. Granulomas were present in the stomach wall, lymph nodes and liver, but the worms were not identified in them. hypersensitivity vasculitis and a bleeding diathesis due to disseminated intravascular coagulation and a circulating anticoagulant caused his death 8 days after the onset of his illness.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
2/517. Thrombosis related to the use of L-asparaginase in adults with acute lymphoblastic leukemia: a need to consider coagulation monitoring and clotting factor replacement.L-asparaginase (L-asp) has become an important component of combination chemotherapy for acute lymphoblastic leukemia (ALL). However, L-asp can produce depletions in many of the clotting factors with an associated risk for thrombosis and hemorrhage. Three consecutive patients seen at the Mayo Clinic with L-asp related thrombosis are described and an in-depth review of the literature is provided. Two of the 3 patients developed central nervous system (CNS) complications with evidence of thrombosis and hemorrhagic infarction. Two of the patients also developed extensive upper extremity thrombosis. The results of comprehensive hemostatic surveys showed marked abnormalities in all 3 patients. Many of the thrombotic complications related to L-asp involve the CNS, as illustrated in 2 of our patients. These patients should be treated aggressively since full recovery is possible. The precise cause of thrombosis is yet to be determined but is likely multifactorial. The optimal treatment and prevention of thrombosis in this group of patients remains poorly defined.- - - - - - - - - - ranking = 4keywords = coagulation (Clic here for more details about this article) |
3/517. Familial thrombophilia and the prothrombin 20210A mutation: association with increased thrombin generation and unusual thrombosis.The 20210A prothrombin mutation has recently been associated with an increased risk of venous thrombosis, but the mechanism of the increased thrombotic risk in affected persons has not been elucidated. We report on a thrombophilic family in which the proband presented with cerebral vein thrombosis and homozygosity for the 20210A prothrombin mutation as her only identifiable risk factor for venous thrombosis. Extended genotyping of family members revealed seven other affected, but asymptomatic, first-degree relatives (one A/A homozygote and six G/A heterozygotes). Plasma levels of prothrombin, prothrombin fragments 1 2 and thrombin-antithrombin complexes were highest in A/A homozygotes, intermediate in G/A heterozygotes and lowest in those with the G/G homozygous normal genotype, while D-dimer levels were elevated only in A/A homozygotes. Our results suggest that the 20210A prothrombin mutation is associated with activation of coagulation and increased thrombin generation, not only in patients with a past history of thrombosis but also in otherwise healthy asymptomatic persons. In a similar fashion to the homozygous factor v Leiden mutation, patients with the homozygous 20210A prothrombin mutation could be at highest risk of thrombosis, as suggested by our patient who presented with unusual thrombosis.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
4/517. FK506-associated thrombotic microangiopathy: report of two cases and review of the literature.BACKGROUND: FK506 is a recently developed immunosuppressant that has been useful in improving the survival of transplanted organs. Among the numerous adverse side effects of FK506, thrombotic microangiopathy (TMA) stands out as an infrequent but severe complication. methods: We report two cases of FK506-associated TMA and review the 19 previous reported cases. RESULTS: From these 21 cases, the reported incidence of FK506-associated TMA is between 1% and 4.7%. It is more frequent in females, and the mean age at presentation is 47 years. Eighty-one percent of the cases occurred in patients with kidney allografts, and the remaining patients had liver, heart, or bone marrow transplants. Clinically, TMA was diagnosed at an average interval of 9.3 months from the time of transplantation. patients may be asymptomatic or may present with the full-blown picture of hemolytic uremic syndrome. All patients had an elevated serum creatinine level but did not always show signs of hemolysis. Trough levels of FK506 were not predictive for the development of TMA, but generally a reduction of drug dose correlated with kidney function improvement and disappearance of the hemolytic picture. The renal allograft biopsy provided a conclusive diagnosis in all 17 cases in which this procedure was performed. Treatment, which mainly consisted of reduction or discontinuation of FK506, anticoagulation, and/or plasmapheresis with fresh-frozen plasma exchange, resolved TMA in most patients (57%). However, in one of these patients (5%), the graft was subsequently lost due to causes unrelated to TMA, such as acute or chronic rejection. Despite treatment, one patient (5%) lost the graft due to acute rejection and persistent TMA, and three other patients (14%) who had bone marrow, heart, and liver transplants, died of multiple organ failure, probably unrelated to TMA. In the remaining four patients (19%), response to treatment was not reported. CONCLUSIONS: TMA must be considered in organ transplant patients treated with FK506 whenever kidney function deteriorates, even in the absence of microangiopathic hemolytic anemia. Although TMA usually responds to treatment, it may, in rare cases, lead to loss of kidney function or even the patient's death.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
5/517. Thrombosis of mitral valve prosthesis presenting as abdominal pain.A 67-year-old woman presented with abdominal pain, anemia, and leukocytosis. Five years previously, the patient had undergone mitral valve replacement with a St. Jude bileaflet mechanical prosthesis. After her admission, echocardiography confirmed an immobile leaflet of the prosthetic valve. At urgent surgery, thrombosis and pannus, obstructing the disc, were found, and the mechanical valve was replaced with a bioprosthesis. The incidence of mitral valve thrombosis is low, ranging from 0.1% to 5.7% per patient per year. patients who receive inadequate anticoagulation, particularly with valve prostheses in the mitral position, have an increased risk for thrombus or pannus formation. Presentation varies, from symptoms of congestive heart failure or systemic embolization, to fever or no symptoms. New or worsening symptoms in a patient with a prosthetic heart valve should raise concerns about prosthetic dysfunction. Aggressive investigation and, if indicated, urgent or emergency surgery for treatment can be lifesaving.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
6/517. Pacemaker-induced superior vena cava obstruction: bypass using the intact azygous vein.Superior vena cava thrombosis due to pacemaker leads is an uncommon but well-recognized complication. Its pathogenesis remains unclear and it is usually a benign condition. Superior vena cava occlusion can be successfully treated by thrombolysis and anticoagulation if the occlusion is recent, balloon venoplasty and stenting, and surgery. We describe a case of superior vena cava obstruction successfully bypassed using the intact native azygous vein, a technique that has not been described before, with excellent long-term results.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
7/517. Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn.This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin iii deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
8/517. Familial overexpression of beta antithrombin caused by an Asn135Thr substitution.We have investigated the basis of antithrombin deficiency in an asymptomatic individual (and family) with borderline levels (approximately 70% antigen and activity) of antithrombin. Direct sequencing of amplified dna showed a mutation in codon 135, AAC to ACC, predicting a heterozygous Asn135Thr substitution. This substitution alters the predicted consensus sequence for glycosylation, Asn-X-Ser, adjacent to the heparin interaction site of antithrombin. The antithrombin isolated from plasma of the proband by heparin-sepharose chromatography contained amounts of beta antithrombin (the very high affinity fraction) greatly increased (approximately 20% to 30% of total) above the trace levels found in normals. Expression of the residue 135 variant in both a cell-free system and COS-7 cells confirmed altered glycosylation arising as a consequence of the mutation. Wild-type and variant protein were translated and exported from COS-7 cells with apparently equal efficiency, in contrast to the reduced level of variant observed in plasma of the affected individual. This case represents a novel cause of antithrombin deficiency, removal of glycosylation concensus sequence, and highlights the potentially important role of beta antithrombin in regulating coagulation.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
9/517. Hypercoagulability in a patient with hypodysfibrinogenemia: implications for clinical management.Dysfibrinogenemia accounts for approximately 0.7% of thrombophilia in patients with venous thromboembolic disease. In 20% of these patients, plasma thrombophilic dysfibrinogen is below 1.0 mg/ml, defining hypodysfibrinogenemia. We describe a young female patient, in whom hypodysfibrinogenemia was the cause of several severe thromboembolic events which occurred even under oral anticoagulation monitored by a standard prothrombin time (PT) test. In this patient, the standard PT test according to Quick underestimated the plasma coagulability in vivo, presumably due to the low levels of dysfunctional fibrinogen as the substrate of the thromboplastin reagent. A PT test supplemented with bovine plasma fibrinogen (Thrombotest) revealed lower fibrinogen-independent international normalized ratio (INR) values in the proposita on oral anticoagulation compared to a control group with eufibrinogenemia. Monitoring therapy with the fibrinogen-independent Thrombotest secured safe anticoagulation in this patient. We suggest to consider PT tests with exogenous fibrinogen (e.g. Thrombotest) to monitor oral anticoagulation in the rare thrombophilic patients with hypodysfibrinogenemia.- - - - - - - - - - ranking = 4keywords = coagulation (Clic here for more details about this article) |
10/517. Right ventricular thrombosis early after bidirectional Glenn shunt.Thrombosis in the right ventricle occurred early after bidirectional superior cavopulmonary shunt in 2 patients with pulmonary atresia with intact ventricular septum and major right ventricular coronary artery communication, and perioperative brain infarction occurred in 1 patient. Clinicians should be aware of the hazards of this potentially lethal complication, and transfusion of platelets and fresh plasma should be minimized. Although the hemodynamic state is good, echocardiography should be performed frequently and strict anticoagulation should be started as early as possible.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
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