1/31. Acquired hemophilia masked by warfarin therapy.People without hemophilia but with autoantibodies specifically directed against the procoagulant activity of factor viii are known to have acquired hemophilia. The bleeding diathesis in these patients is often severe and life-threatening. The definite laboratory diagnosis of this disorder includes demonstration of low factor viii levels in plasma with a high titer of factor viii inhibitors, but the initial suspicion for its presence should rise in view of a prolonged partial thromboblastin time (PTT) and a normal prothrombin time associated with an acquired bleeding disorder. Oral anticoagulant treatment is known to prolong PTT as well, and the merger of these 2 situations may cause delayed diagnosis of acquired hemophilia with devastating consequences. We describe here the first reported case of acquired hemophilia diagnosed in a patient treated with warfarin. In such patients prolonged PTT may be ascribed to warfarin therapy rather than to acquired hemophilia, thus causing a dangerous delay in diagnosis.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
2/31. Gingival bleeding, epistaxis and haematoma three days after gastroenteritis: the haemorrhagic lupus anticoagulant syndrome.A 3 year and 9 month-old girl presented with gingival bleeding, epistaxis, and multiple haematomas 3 days after an acute episode of gastroenteritis. prothrombin time and activated partial thromboplastin time were prolonged with reduced clotting activity of factor II (< 10%), VIII (<1%), IX (3%), XII (10%) and evidence of a high titre inhibitor. Prothrombin (factor II) level was below the detection limit, both in a functional and immunological assay. It did not increase after administration of vitamin k or fresh frozen plasma. Further studies revealed presence of a strong lupus anticoagulant and a specific IgG antibody against prothrombin. factor viii antigen levels also were reduced (31%), but to a lesser extent than functionally determined factor viii (<1%). blood coagulation normalised following clinical recovery 6 weeks after admission. The pathophysiology of this acquired inhibitor phenomenon (accelerated clearance of complexes of clotting factors and phospholipids) is discussed. CONCLUSION: The haemorrhagic lupus anticoagulant syndrome (acquired hypoprothrombinaemia lupus anticoagulant syndrome) is a rare presentation of acquired bleeding diathesis in childhood. Since most cases in post-infectious children are asymptomatic, it might be underdiagnosed. In children with newly appearing bleeding symptoms or unclear prolonged prothrombin time or activated partial thromboplastin time, one has to consider this syndrome which could lead to relevant bleeding.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
3/31. Molecular and functional characterization of a natural homozygous Arg67His mutation in the prothrombin gene of a patient with a severe procoagulant defect contrasting with a mild hemorrhagic phenotype.In a patient who presented with a severe coagulation deficiency in plasma contrasting with a very mild hemorrhagic diathesis a homozygous Arg67His mutation was identified in the prothrombin gene. Wild-type (factor IIa [FIIa]-WT) and mutant Arg67His thrombin (FIIa-MT67) had similar amidolytic activity. By contrast, the k(cat)/K(m) value of fibrinopeptide a hydrolysis by FIIa-WT and FIIa-MT67 was equal to 2.1 x 10(7) M(-1)s(-1) and 9 x 10(5) M(-1)s(-1). Decreased activation of protein c (PC) correlated with the 33-fold decreased binding affinity for thrombomodulin (TM; K(d) = 65.3 nM vs 2.1 nM, in FIIa-MT67 and in FIIa-WT, respectively). In contrast, hydrolysis of PC in the absence of TM was normal. The Arg67His mutation had a dramatic effect on the cleavage of protease-activated G protein-coupled receptor 1 (PAR-1) 38-60 peptide (k(cat/)K(m) = 4 x 10(7) M(-1)s(-1) to 1.2 x 10(6) M(-1)s(-1)). FIIa-MT67 showed a weaker platelet activating capacity, attributed to a defective PAR-1 interaction, whereas the interaction with glycoprotein Ib was normal. A drastic decrease (up to 500-fold) of the second-order rate constant pertaining to heparin cofactor ii (HCII) interaction, especially in the presence of dermatan sulfate, was found for the FIIa-MT67 compared with FIIa-WT, suggesting a severe impairment of thrombin inhibition by HCII in vivo. Finally, the Arg67His mutation was associated with a 5-fold decrease of prothrombin activation by the factor xa-factor va complex, perhaps through impairment of the prothrombin-factor va interaction. These experiments show that the Arg67His substitution affects drastically both the procoagulant and the anticoagulant functions of thrombin as well as its inhibition by HCII. The mild hemorrhagic phenotype might be explained by abnormalities that ultimately counterbalance each other.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
4/31. Selective mediastinal tamponade to control coagulopathic bleeding.End-stage congestive heart failure in combination with major cardiac surgery may cause severe postoperative bleeding due to coagulopathy. Bleeding diathesis can be very difficult to control despite careful surgical hemostasis and aggressive medical treatment. We describe a technique that involves the use of negative pressure in the mediastinum to control severe postoperative bleeding after cardiac surgery.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
5/31. Persistent coagulopathy in snake bite.Bleeding diathesis is a cardinal feature of viperine bite, which has been thought to last not more than 24 hours. There is scarcity of literature about prolonged bleeding disorder in snake envenomation. Various explanations suggested in the literature include-temporary decrease in antivenin levels, rapid elimination of antivenin from circulation or continuous release of unneutralised venom from the envenomated site. Two children with prolonged coagulopathy lasting for more than a week, correction of which required more than 300 ml of antisnake venom are reported here.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
6/31. Primary fibrinogenolysis complicating second trimester placental separation.We present a case of second trimester placental separation complicated by severe bleeding diathesis. Primary fibrinogenolysis is suggested as the cause of the coagulopathy.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
7/31. cytomegalovirus-associated bleeding diathesis in renal transplant recipients.cytomegalovirus (CMV) infection is reported to be capable of modifying endothelial surface with subsequent increased risk of thromboembolic complications. Nevertheless, there are only sparse reports on its role in the development of bleeding diathesis. Here we report two renal transplant recipients who manifested severe coagulation disorders associated with acute CMV infection. Antiviral therapy was followed by consistent correction of coagulation abnormalities.- - - - - - - - - - ranking = 5keywords = diathesis (Clic here for more details about this article) |
8/31. Severe coagulopathy after a bite from a 'harmless' snake (Rhabdophis subminiatus).European physicians may occasionally be confronted with cases of snake venom poisoning. The increasing interest in snakes as pets has unfortunately resulted in the importation of a number of venomous snakes. We here report the third known case in the medical literature of a patient who was exposed to severe coagulopathy after being bitten by a 'harmless' ringsnake (Rhabdophis subminiatus). The prolonged bleeding diathesis and the complete depletion of fibrinogen observed in our patient are consistent with the presence of a factor X activator in the venom, as has been described in a murine model. In agreement with the two previous case reports, we found no evidence of severe organ damage, despite active intravascular coagulation.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
9/31. Prothrombinex-induced thrombosis and its management with regional plasminogen activator in hepatic failure.Fulminant hepatic failure causes a bleeding diathesis as a result of impaired synthesis of hepatic clotting factors, thrombocytopenia, fibrinolysis and disseminated intravascular coagulation. Administration of clotting factor concentrates can cause thrombosis in patients with acute hepatic failure. Regional infusion of recombinant tissue-type plasminogen activator may be used to induce local thrombolysis. A case report of a five-year-old child is presented and the literature is reviewed.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
10/31. Elevated urokinase-type plasminogen activator level and bleeding in amyloidosis: case report and literature review.Hyperfibrinolytic states are reported to be a cause of bleeding in patients with amyloidosis. We reviewed the literature on excessive fibrinolysis in association with amyloidosis and report our findings from a patient with idiopathic amyloidosis who developed a bleeding diathesis. Coagulation laboratory studies indicated elevated plasminogen activator levels associated with a reduction of plasminogen and alpha 2-plasmin inhibitor (alpha 2-PI) levels. The level of tissue-type plasminogen activator (t-PA) inhibitor and t-PA antigen were normal. However, the patient did have a five- to sevenfold increase in amidolytic activity for the urokinase substrate pyro-Glu-Gly-Arg-pNA (S-2444). This case therefore represents a novel example of a hyperfibrinolytic state associated with amyloidosis caused by elevated urokinase-type plasminogen activator (u-PA). Epsilon-amino caproic acid (EACA) therapy resulted in an increase in alpha 2-PI and plasminogen levels and effectively reduced the blood loss. Hyperfibrinolytic states in amyloidosis have now been reported to be due to elevated t-PA and u-PA and depleted t-PA inhibitor.- - - - - - - - - - ranking = 1keywords = diathesis (Clic here for more details about this article) |
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