Cases reported "Thrombophilia"

Filter by keywords:



Filtering documents. Please wait...

1/10. budd-chiari syndrome associated with factor v leiden mutation: a report of 6 patients.

    budd-chiari syndrome is characterized by hepatic venous outflow obstruction. Although myeloproliferative disorders are usually responsible for this severe thrombotic disorder, deficiency or dysfunction of the natural anticoagulants can be involved. Resistance to activated protein C caused by factor v Leiden mutation has been recently identified as a major cause of thrombophilia. We report 6 patients with budd-chiari syndrome associated with factor v Leiden mutation combined with another acquired thrombophilic state (myeloproliferative disorder and lupus anticoagulant in 3 cases) and without another thrombophilic disorder in the other 3 cases. We conclude that factor v Leiden mutation should be evaluated in any case of hepatic vein occlusion because the prevalence of this mutation in the general population is high.
- - - - - - - - - -
ranking = 1
keywords = hepatic, obstruction
(Clic here for more details about this article)

2/10. budd-chiari syndrome in a patient with factor v Leiden--successful treatment by TIPSS placement followed by liver transplantation.

    The causes of budd-chiari syndrome (BCS) comprise several diseases leading to thrombophilia. One of the most common thrombophilic disorders is resistance against activated protein C, caused by a single point mutation of the factor v gene. In December 1993, a 22-year-old patient was given a diagnosis of subacute BCS with occlusion of all major hepatic veins. Placement of a transjugular intrahepatic portosystemic stent shunt led to rapid disappearance of ascites and hepatic encephalopathy. During the following two years, recurrent partial occlusions of the shunt were treated by balloon angioplasty. The cause of the BCS still being unknown, in October 1996 we performed extensive laboratory investigations concerning states of thrombophilia and found moderately elevated IgG anticardiolipin antibodies (19.7 U/ml) and a resistance against activated protein C caused by heterozygosity for a point mutation of the factor v gene (1691G-->A; factor v Leiden). As a consequence, oral anticoagulation with coumarin was initiated. In October 1997, elective liver transplantation was performed which led to disappearance of APC resistance. Moreover, IgG anticardiolipin antibodies have been negative since then. If BCS is caused by APC resistance, liver transplantation not only treats the chronic liver disease but also cures the state of thrombophilia since factor v is mainly synthesized in the liver.
- - - - - - - - - -
ranking = 1.1122767664909
keywords = hepatic
(Clic here for more details about this article)

3/10. Erythematous plaques due to platelet plugging: a clue to underlying myeloproliferative disorder.

    We report a syndrome in a middle-aged woman characterized by tender erythematous plaques with histologic evidence of dramatic dermal vessel occlusion. These cutaneous findings occurred in association with progressive inferior vena cava and portal vein thrombosis while on coumarin anticoagulation, following hepatic transplantation for budd-chiari syndrome. The material occluding dermal vessels was proven by immunohistochemical staining to be platelet plugs. These findings led to the diagnosis of an underlying myeloproliferative disorder explaining both her cutaneous and liver abnormalities and institution of appropriate platelet directed anticoagulation with aspirin.
- - - - - - - - - -
ranking = 0.37075892216363
keywords = hepatic
(Clic here for more details about this article)

4/10. Acute budd-chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein c deficiency.

    We report the case of a female patient who had severe thrombotic complications in peripheral (V. jugularis, subclavia, brachialis, poplitea) and visceral (portal and splenic) veins 4 years after the first diagnosis of severe ulcerative pancolitis. A thrombolysis therapy for subclavian and jugular vein thrombosis was performed without complication, but she soon developed acute thrombosis of the hepatic veins (acute budd-chiari syndrome). She quickly recovered after liver transplantation and now - 6 years later - she lives a normal life with continuous anticoagulation and medical therapy of the colitis.3 possible causes for the severe coagulation defect in this patient can be supposed: thrombocytosis, protein c deficiency and an antiphospholipid antibody syndrome.
- - - - - - - - - -
ranking = 0.37075892216363
keywords = hepatic
(Clic here for more details about this article)

5/10. Dangerous thrombophilic states and internal pathologies: 3 cases of thrombosis of the abdominal veins.

    Thrombosis of the abdominal veins is a rare clinical condition which can be assimilated with the more frequent localization of deep venous thrombosis of the lower limbs. In the last few years great attention has been paid to possible risk factors for thrombosis of the abdominal veins. Two risk factors that have been identified are the presence of internal diseases and congenital and/or acquired abnormalities of haemostasis. The authors describe 3 clinical cases (splenic and portal thrombosis due to congenital thrombophilia, budd-chiari syndrome, portal cavernoma consequent to ovarian neoplasia) with different etiopathogenesis to show how this apparently rare condition is today more frequently encountered and easier to recognize. In the presence of thrombosis of major venous structures the search and the identification of intrinsic internal risk factors and of congenital and acquired thrombophilic disorders remains of great importance. Screening for thrombophilia includes blood C and S proteins, AT III, homocysteine, Leiden mutation of the factor v gene, G20210A mutation of the prothrombin gene, antiphospholipid antibodies. The presence of one or more of these risk factors allows the identification of the cases of portal thrombosis (EHPVO) responsible for about 10% of all the cases of portal hypertension, without cirrhosis or other hepatic lesions. The primary diagnostic procedure however remains color-Doppler ultrasonography which represents the most simple and the cheapest diagnostic investigation for the study of the portal and suprahepatic vein system, but it's strictly operator dependent.
- - - - - - - - - -
ranking = 0.74151784432726
keywords = hepatic
(Clic here for more details about this article)

6/10. Venous claudication in a child with thrombophilia.

    Deep venous thrombosis (DVT) rarely occurs in active children. Its presence usually suggests an inherited or acquired hypercoagulable state. Occasionally mechanical obstruction may be the inciting factor in this process. Initial management usually consists of sequential heparin and warfarin anticoagulation. We present the management of DVT in an adolescent girl with elevated levels of c-reactive protein and lupus anticoagulant. Venous claudication and severe lower-extremity swelling on ambulation complicated her course. After more than 2 weeks of conservative therapy with anticoagulation thrombolytic therapy was instituted. This was terminated early because of mild hematuria. However, follow-up duplex scan at 2 years has shown complete resolution of the iliofemoral thrombosis. Spontaneous DVT in children differ from that in adults in that an underlying etiology can usually be uncovered. These differences are explored.
- - - - - - - - - -
ranking = 0.25848215567274
keywords = obstruction
(Clic here for more details about this article)

7/10. liver transplantation as definitive treatment for a factor v Leiden mutation.

    liver transplantation (LT) was achieved for factor v Leiden-induced thrombophilia in a neonate with hepatic veno-occlusive disease. Initial LT was performed with a liver segment removed from a child with primary oxalosis. Four months later, a second, definitive LT was performed. The child remains well without recurrent thrombosis.
- - - - - - - - - -
ranking = 0.37075892216363
keywords = hepatic
(Clic here for more details about this article)

8/10. Recurrent thrombotic occlusion of a transjugular intrahepatic portosystemic stent-shunt due to activated protein c resistance.

    The transjugular intrahepatic portosystemic stent-shunt (TIPS) has successfully been used in the management of refractory variceal bleeding and ascites in patients with portal hypertension. Major drawbacks are the induction of hepatic encephalopathy and shunt dysfunction. We present a 59-year-old woman with alcoholic liver cirrhosis who received a TIPS because of recurrent bleeding from esophageal varices. Stent occlusion occurred 4 mo after placement of the TIPS. Laboratory testing revealed resistance to activated protein C (APC). Combination therapy with low-dose enoxaparin and clopidogrel could not prevent her recurrent stent occlusion. Finally, therapy with high-dose enoxaparin was sufficient to prevent further shunt complications up to now (follow-up period of 1 year). In conclusion, early occlusion of a TIPS warrants testing for thrombophilia. If risk factors are confirmed, anticoagulation should be intensified. There are currently no evidence-based recommendations regarding the best available anticoagulant therapy and surveillance protocol for patients with TIPS.
- - - - - - - - - -
ranking = 2.2245535329818
keywords = hepatic
(Clic here for more details about this article)

9/10. budd-chiari syndrome as the first manifestation of polycythemia vera in young women with inherited thrombophilic state: an aggressive form of myeloproliferative disorder requiring multidisciplinary management.

    The budd-chiari syndrome (BCS), characterized by the obstruction and occlusion of the suprahepatic veins, is a rare but typical complication occurring in patients with polycythemia vera (PV). We describe three young women who developed BCS as first manifestation of PV, in association with an inherited thrombophilic state and in the absence of concomitant use of oral contraceptives. Our report illustrates the existence of an aggressive form of myeloproliferative disorder, which requires prompt recognition and immediate therapeutic intervention including cytostatic drugs and anticoagulant treatment. Furthermore, we suggest the need of routine screening for thrombophilic state in young women affected by PV.
- - - - - - - - - -
ranking = 0.62924107783637
keywords = hepatic, obstruction
(Clic here for more details about this article)

10/10. Non-malignant bone marrow necrosis: a report of two cases.

    We present two cases of bone marrow necrosis not associated with malignancy, infection or sickle cell disease. The first case, a 28 year old woman with the antiphospholipid syndrome and a factor v Leiden abnormality, suffered an illness characterised by multiple organ thromboses, anemia and refractory thrombocytopenia. She had documented bone marrow necrosis of the posterior iliac spine and numerous hot spots on bone scanning suggestive of widespread marrow necrosis. This patient also suffered hepatic infarcts and a miscarriage and may represent an explanation for the previously described "catastrophic antiphospholipid syndrome". The second patient developed widespread bone pain over a three week period, underwent a cholecystectomy and suffered major post-operative complications including a delayed transfusion reaction and disseminated intravascular coagulation. pancytopenia developed and bone marrow trephines from numerous foci revealed widespread bone marrow necrosis. The only predisposing factor to account for this presentation was that the patient had been sniffing glue for two months prior to the illness, as the foci of necrosis had healed on repeat marrow examination eight weeks later when the patient had abstained from glue sniffing. This case may represent a reversible, toxic cause of bone marrow necrosis.
- - - - - - - - - -
ranking = 0.37075892216363
keywords = hepatic
(Clic here for more details about this article)
| Next ->


Leave a message about 'Thrombophilia'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.