Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report

Bittencourt, Maraya de Jesus Semblano; Dias, Carolina Moraes; Lage, Thaiane Lima; Barros, Renata Silva; Paz, Otávio Augusto Gomes da; Vieira, Waldonio de Brito

doi:10.1590/abd1806-4841.20131930

Cited by 6 publications

(5 citation statements)

References 7 publications

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“…Strong positive test results for antiphospholipid antibodies were present in 3 patients. TS has also been reported in association with an idiopathic hypereosinophilic syndrome,15 inflammatory bowel disease,16 Kimura disease,17 and Behcet disease 18. In our case, no definite precipitating event and none of the underlying prothrombotic states described above were identified.…”

Section: Discussionsupporting

confidence: 56%

Successful Management of Catastrophic Thrombotic Storm in a Young Boy: A Case Report From Northern India

Jindal

Ahluwalia

Suku

et al. 2021

Journal of Pediatric Hematology/Oncology

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Thrombotic storm is a rare clinical entity characterized by acute to subacute thrombosis developing at multiple sites over a few days to a few weeks. An 11-year-old boy presented with headache and facial nerve palsy. He was found to have cortical sinus venous thrombosis and was initiated on low molecular weight heparin, but rapidly progressed with thromboses involving the pulmonary arteries and deep veins of the legs. Thereafter managed on high-dose unfractionated heparin, he eventually stabilized after a hospital stay of 34 days. Genetic analysis showed potentially pathogenic variants in the factor V and stabilin-2 genes.

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Section: Discussionsupporting

confidence: 56%

Successful Management of Catastrophic Thrombotic Storm in a Young Boy: A Case Report From Northern India

Jindal

Ahluwalia

Suku

et al. 2021

Journal of Pediatric Hematology/Oncology

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“…The most common causes are hypercoagulation states [ 21 ]. The frequency of vascular involvement (superficial and deep venous thrombosis, arterial aneurysms, and occlusions) in BD ranges from 7% to 29% [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

“…Incidence of BCS in patients with BD was reported at between 1.1% and 5.8% [ 7 , 20 , 24 , 25 ]. Uskudar et al [ 22 ] reported in their series of 75 patients that web was 16%, hydatid cyst was 12%, and BD was 9% responsible in BCS etiology in Turkey.…”

Section: Discussionmentioning

confidence: 99%

Colon Perforation and Budd-Chiari Syndrome in Behçet’s Disease

et al. 2015

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Patient: Female, 38Final Diagnosis: Behçet’s diseaseSymptoms: Severe abdominal pain • feverMedication: —Clinical Procedure: Parsiyel colectomySpecialty: SurgeryObjective:Unusual clinical courseBackground:Behçet’s disease is a chronic inflammatory disease involving multiple systems, with vasculitis being the most important pathological feature. Multiple colon perforations are thought to be secondary to vasculitis and they occur in patients with ulcers. These may be encountered within the entire colon but most commonly in the ileocecal region. Intestinal perforation and Budd-Chiari syndrome are infrequent in Behçet’s disease, and are associated with high mortality and morbidity. Budd-Chiari syndrome results from occlusion of either hepatic veins or adjacent inferior vena cava, or both.Case Report:We report a patient with Behçet’s disease having multiple perforations in the transverse colon, descending colon, and sigmoid colon. The patient also had Budd-Chiari syndrome due to inferior vena cava thrombosis extending into the right and middle hepatic vein. Our observations are presented with a review of the literature.Conclusions:In Behçet’s disease, treatment of colon perforation necessitates urgent surgery, whereas management of Budd-Chiari syndrome is directed towards the underlying cause. Behçet’s disease, as a chronic multisystemic disease with various forms of vasculitis, is resistant to medical and surgical treatment. Prognosis is worse in Behçet’s disease with colon perforation than that in Budd-Chiari syndrome alone.

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“…60,61 Behçet disease is associated with an increased risk for VTE, and, to a lesser extent, arterial occlusions and aneurysms. 62 The most common thrombotic complication in patients with Behçet disease is DVT, and patients frequently have recurrent events.…”

Section: Other Disordersmentioning

confidence: 99%

How I treat catastrophic thrombotic syndromes

Ortel

Erkan

Kitchens

2015

Blood

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Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thrombocytopenic purpura (TTP) or heparin-induced thrombocytopenia (HIT), and Trousseau syndrome, but some patients present with multiple thrombotic events in the absence of associated prothrombotic disorders. Diagnostic workup must rapidly determine which, if any, of these syndromes are present because therapeutic management is driven by the underlying disorder. With the exception of atypical presentations of TTP, which are treated with plasma exchange, anticoagulation is the most important therapeutic intervention in these patients. Effective anticoagulation may require laboratory confirmation with anti–factor Xa levels in patients treated with heparin, especially if the baseline (pretreatment) activated partial thromboplastin time is prolonged. Patients with catastrophic APS also benefit from immunosuppressive therapy and/or plasma exchange, whereas patients with HIT need an alternative anticoagulant to replace heparin. Progressive thrombotic events despite therapeutic anticoagulation may necessitate an alternative therapeutic strategy. If the thrombotic process can be controlled, these patients can recover, but indefinite anticoagulant therapy may be appropriate to prevent recurrent events.

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Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report

Cited by 6 publications

References 7 publications

Successful Management of Catastrophic Thrombotic Storm in a Young Boy: A Case Report From Northern India

Successful Management of Catastrophic Thrombotic Storm in a Young Boy: A Case Report From Northern India

Colon Perforation and Budd-Chiari Syndrome in Behçet’s Disease

How I treat catastrophic thrombotic syndromes

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