scholarly journals Venous Thrombosis in Acquired Hemophilia: The Complex Management of Competing Pathologies

TH Open ◽  
2019 ◽  
Vol 03 (04) ◽  
pp. e325-e330 ◽  
Author(s):  
Manu Chhabra ◽  
Zhen Wan Stephanie Hii ◽  
Joseph Rajendran ◽  
Kuperan Ponnudurai ◽  
Bingwen Eugene Fan

Abstract Introduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major surgery, or in association with the administration of FVIII concentrate and activated prothrombin complex concentrates, but occurrence of spontaneous DVT is even more uncommon. Aim We describe the challenging management of extensive DVT in a patient with acquired hemophilia A with concurrent hemorrhagic manifestations and review similar published cases. Methods We summarize a series of 10 cases with the following demographics: 6 males and 4 females; median age at presentation of 65 (21–80); mean inhibitor titer of 68.5 Bethesda Units (BU 1.9 to BU 350). Results Four cases were idiopathic and six had associated conditions (cancer [two cases], recent pregnancy [two cases], and recent surgery [two cases]). Three cases had an inferior vena cava filter inserted for acute lower limb DVT/pulmonary embolism. Inhibitor eradication was achieved with high-dose steroids with or without cyclophosphamide, and adjunct Rituximab administration was used in three cases. One patient received concurrent therapeutic plasma exchange (TPE). Inhibitor eradication was fastest with concurrent TPE at 6 days (range: 6–733 days). The 30-day survival was 90%. Conclusions There was adequate response of inhibitors to immunosuppression with steroids and cyclophosphamide therapy. For more refractory disease, Rituximab is emerging as a beneficial and cost-effective adjunct with better rates of complete remission, and the threshold for its use may be lowered in this complex cohort with dual competing pathologies.

2020 ◽  
Vol 9 (11) ◽  
pp. 3509
Author(s):  
Zachary Liederman ◽  
Noel Chan ◽  
Vinai Bhagirath

In patients with suspected venous thromboembolism, the goal is to accurately and rapidly identify those with and without thrombosis. Failure to diagnose venous thromboembolism (VTE) can lead to fatal pulmonary embolism (PE), and unnecessary anticoagulation can cause avoidable bleeding. The adoption of a structured approach to VTE diagnosis, that includes clinical prediction rules, D-dimer testing and non-invasive imaging modalities, has enabled rapid, cost-effective and accurate VTE diagnosis, but problems still persist. First, with increased reliance on imaging and widespread use of sensitive multidetector computed tomography (CT) scanners, there is a potential for overdiagnosis of VTE. Second, the optimal strategy for diagnosing recurrent leg deep venous thrombosis remains unclear as is that for venous thrombosis at unusual sites. Third, the conventional diagnostic approach is inefficient in that it is unable to exclude VTE in high-risk patients. In this review, we outline pragmatic approaches for the clinician faced with difficult VTE diagnostic cases. In addition to discussing the principles of the current diagnostic framework, we explore the diagnostic approach to recurrent VTE, isolated distal deep-vein thrombosis (DVT), pregnancy associated VTE, subsegmental PE, and VTE diagnosis in complex medical patients (including those with impaired renal function).


1987 ◽  
Author(s):  
A Elias ◽  
J L Bouvier ◽  
G Le Corff ◽  
A Serradimigni

The sucess of the fibrinolytic or surgical treatment of venous thrombosis depends largely on how old the thrombosis is.To study the therapeutic predictibility of the ultrasonographic signs,their pretherapeutic aspects were compared to the results of the fibrinolytic treatment during 3 days or to the anatomic aspects intra-operatively. This prospective study was carried out on 21 patients presenting an isolated venous thrombosis confirmed by venography. 65 involved venous segments from the popliteal to the inferior vena cava were studied.The following are analyzed : 1) The C.W. Doppler signal when there is a complete obstruction of the femoral iliac junction : absence or presence of flow (MEDAS0NICS BF4A 5.3MHZ).2)The morphological and dynamic aspects of the vein, the thrombus and the flow using echographic test (DIASONICS DRF 400. 10-7.5-5MHZ) were retained as predictible signs of sucessfull treatment a hypodens^homogeneous thrombus with major venous dilation (about twice the controlateral vein) where there is a total obstruction, and a free floating thrombus or even “elastic”,soft,compressible when there is a partial obstruction.The opposite signs were interpreted as predicting a therapeutic failure : a dense heterogeneous adherent and hard thrombus.The criteria for thrombolysis efficiency is the total disappearance of the thrombus when there was a partial obstruction,or a recanalization (>50%)where there was a complete occlusion.The ultrasonographic predictible signs compared with the effective results are indicated in the following tables.Ultrasonography enables most cases tne recognition or a “recent” thrombus from an “old” one and allows talcing thus a cal-lated risk. If the evaluation of the echotexture of the thrombus is subjective and technique dependant, and if an objective measurement of the density distribution insight the clot as reported is preferable, a combined study of the other ultrasonographic parameters seems to be essential.


2018 ◽  
Author(s):  
Albeir Y Mousa

Acute deep venous thrombosis (DVT) of iliofemoral segment is one of the most dreaded presentations of venous thromboembolism, as it can not only compromise the function of the extremity but may also result in pulmonary embolism and even death. There are many causes for acute iliofemoral DVT, including underdiagnosed May-Thurner syndrome, hypercoagulable syndrome, and external compression on iliocaval segment. The available treatment depends on the acuity of the symptoms. Acute iliofemoral DVT can be treated with medical anticoagulation, pharmacomechanical therapy, including thrombolysis or surgical thrombectomy. Chronic iliofemoral occlusion may be treated with recanalization of the occluded segments with angioplasty stenting. This review contains 4 Figures, 4 Tables and 63 references Key Words: acute, angioplasty, deep venous thrombosis, iliofemoral, inferior vena cava, pharmacomechanical therapy, occlusion, stent


2018 ◽  
Author(s):  
Albeir Y Mousa

Acute deep venous thrombosis (DVT) of iliofemoral segment is one of the most dreaded presentations of venous thromboembolism, as it can not only compromise the function of the extremity but may also result in pulmonary embolism and even death. There are many causes for acute iliofemoral DVT, including underdiagnosed May-Thurner syndrome, hypercoagulable syndrome, and external compression on iliocaval segment. The available treatment depends on the acuity of the symptoms. Acute iliofemoral DVT can be treated with medical anticoagulation, pharmacomechanical therapy, including thrombolysis or surgical thrombectomy. Chronic iliofemoral occlusion may be treated with recanalization of the occluded segments with angioplasty stenting. This review contains 4 Figures, 4 Tables and 63 references Key Words: acute, angioplasty, deep venous thrombosis, iliofemoral, inferior vena cava, pharmacomechanical therapy, occlusion, stent


2016 ◽  
Vol 62 (2) ◽  
pp. 266-268 ◽  
Author(s):  
Carmen Duicu ◽  
Gabriela Bucur ◽  
Iunius Simu ◽  
Florin Tripon ◽  
Oana Marginean

AbstractCongenital inferior vena cava anomalies have a reduced frequency in general population, many times being an asymptomatic finding. Patients caring such anomalies are at risk to develop deep vein thrombosis. In this paper, we present 2 siblings with deep venous thrombosis and inferior vena cava abnormalities, with a symptomatic onset at similar age. The inferior vena cava abnormality was documented by an angio-CT in each case. The thrombophilic workup was negative. Patients were treated with conservative therapy: low molecular weight heparin anticoagulants converted later to oral anticoagulant with resolution of symptoms and disappearance of the thrombus. Finally, in the absence of any risk factor in a young patient admitted with deep vein thrombosis investigations to exclude inferior vena cava anomalies are mandatory.


2011 ◽  
Vol 2011 ◽  
pp. 1-11 ◽  
Author(s):  
Andrew D. Blann ◽  
Simon Dunmore

The most frequent ultimate cause of death is myocardial arrest. In many cases this is due to myocardial hypoxia, generally arising from failure of the coronary macro- and microcirculation to deliver enough oxygenated red cells to the cardiomyocytes. The principle reason for this is occlusive thrombosis, either by isolated circulating thrombi, or by rupture of upstream plaque. However, an additionally serious pathology causing potentially fatal stress to the heart is extra-cardiac disease, such as pulmonary hypertension. A primary cause of the latter is pulmonary embolus, considered to be a venous thromboembolism. Whilst the thrombotic scenario has for decades been the dominating paradigm in cardiovascular disease, these issues have, until recently, been infrequently considered in cancer. However, there is now a developing view that cancer is also a thrombotic disease, and notably a disease predominantly of the venous circulation, manifesting as deep vein thrombosis and pulmonary embolism. Indeed, for many, a venous thromboembolism is one of the first symptoms of a developing cancer. Furthermore, many of the standard chemotherapies in cancer are prothrombotic. Accordingly, thromboprophylaxis in cancer with heparins or oral anticoagulation (such as Warfarin), especially in high risk groups (such as those who are immobile and on high dose chemotherapy), may be an important therapy. The objective of this communication is to summarise current views on the epidemiology and pathophysiology of arterial and venous thrombosis in cancer.


2016 ◽  
Vol 36 (suppl_1) ◽  
Author(s):  
Satish Singh ◽  
Aiilyan K Houng ◽  
Samantha Howard ◽  
B Tyler Emerson ◽  
Guy L Reed

Introduction: Deep venous thrombosis is a major cause of death and disability. Despite anticoagulation treatment, venous thrombi persist for months, causing chronic venous obstruction, inflammatory remodeling and post-thrombotic syndrome. The mechanisms responsible for impaired clearance of venous thrombi are poorly understood. Alpha 2-antiplasmin (a2AP) is the primary physiological inhibitor of plasmin and a key regulator of the fibrinolytic pathway. The role of a2AP in the resolution of venous thrombosis has not been determined. Hypothesis: We tested the hypothesis that a2AP prevents the resolution of experimental deep venous thrombi. Methods and Results: Thrombus resolution and content were examined in a2AP +/+ and a2AP -/- mice using a well-established, fibrinolytic-resistant, stasis model induced by ligation of the inferior vena cava (IVC). Thrombus weight and composition were determined after 7 days. Data was analyzed by one-way ANOVA with Neumann-Keul’s correction. Thrombus weight was reduced in a2AP -/- mice by >90% when compared to a2AP +/+ mice (p<0.001); there was no significant difference between a2AP -/- mice and shams. Histochemical and immunofluorescence staining showed significant reductions in IVC fibrin content, neutrophil recruitment and matrix metalloproteinase-9 expression in a2AP -/- mice (p<0.001) vs. a2AP +/+ mice. The relative effect of plasminogen activation and a2AP on resolution of preformed venous thrombi was examined in wild-type a2AP +/+ mice treated 24 h after IVC ligation with tissue plasminogen activator (TPA) (1.2 or 5 mg/kg) or a monoclonal antibody inactivating a2AP (10 mg/Kg). By comparison to 7 day old venous thrombi in untreated mice, treatment with TPA at 1.2 mg/kg or 5 mg/kg did not decrease thrombus size after 7 days. In contrast, a2AP inactivation significantly reduced thrombus weight vs. untreated and TPA-treated mice (p<0.01 to p<0.001). Conclusions: In experimental venous thrombosis, a2AP was required for the persistence of venous thrombi 7 days after formation. Venous thrombi resisted TPA, but were sensitive to resolution after a2AP inactivation. This suggests that a2AP may be responsible for the persistence of clinical venous thrombosis in humans.


Author(s):  
Susan Mohammadi ◽  
Nastaran Hesam Shariati ◽  
Fardin Fathi ◽  
Siamak Arshadi ◽  
Fahimeh Rajabi ◽  
...  

A lack of congenital Inferior Vena Cava (IVC) is an uncommon malformation that has been identified in combination with idiopathic Deep Venous Thrombosis (DVT), exclusively. It may not even be revealed during the lifetime. A 63-year-old female was accepted with three months of abdominal and pelvic pain and localized edema on the right flank. During this admission, she was examined and recognized for deep vein thrombosis (DVT). Ct scan images showed a lack of the Inferior Vena Cava with enormous thrombosis collaterals of the superficial vein in the abdomen. In this case report, we report a woman with side pain who has an absence of the IVC.  


Author(s):  
Mi Zhou ◽  
Jie Yin

Catheter-related DVT under the setting of TTP or TTP recovery stage may be presented as a more fulminant form. Certain guidelines for anticoagulation and antiplatelet therapy for thrombosis early should be further established. We present here a case of a patient presented with Comb (+) TTP and developed catheter-associated deep vein thrombosis (DVT). The patient was firstly diagnosed with Evans syndrome. However, he was refractory to a methylprednisolone pulse therapy with a combination of platelet transfusion and eventually developed microangiopathy of central nerve system. The immediate start of PEX (1500ml/d) induced a complete remission of acquired TTP and disappearance of neurological signs and symptoms. However, external iliac and femoro-popliteal venous thrombosis was diagnosed subsequently, inferior vena cava filter (IVC) filter was immediately planting accompanied with anticoagulation therapy. Meanwhile, PEX session was sustained as well as oral anticoagulant (rivaroxaban). 14 days later, the patient got full recovery. Our report aims at raising awareness of Catheter-related DVT under the setting of TTP should be cautious. It is necessary to start anticoagulation and antiplatelet therapy for thrombosis early, especially in such cases when PLT count > 50×109/L.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 19-20
Author(s):  
Nirav Dhanesha ◽  
Manish Jain ◽  
Prakash Doddapattar ◽  
Anetta Undas ◽  
Anil K Chauhan

Objective: Obesity is a significant risk factor for deep vein thrombosis (DVT). The mechanisms of increased DVT in preexisting comorbid condition of obesity remain poorly understood. Cellular fibronectin containing extra domain A (Fn-EDA), an endogenous ligand for toll-like-receptor 4 (TLR4), is known to contribute to thrombo-inflammation in the experimental models. However, the role of Fn-EDA in modulation of venous thrombosis in context of obesity is not elucidated yet. Approach and Results: We found that cellular Fn-EDA levels were significantly elevated in plasma of venous thromboembolism (VTE) patients that positively correlated with body mass index (BMI). To investigate whether Fn-EDA promotes venous thrombosis in obese condition, WT and Fn-EDA-/- mice were either fed a control or high-fat diet (HF-diet) for 12-weeks. DVT was induced by inferior vena cava stenosis and evaluated after 48 hours. We found that HF diet-fed WT mice exhibited increased DVT susceptibility compared with control diet-fed WT mice. In contrast, HF-fed Fn-EDA-/- mice exhibited significantly reduced thrombus weight and decreased incidence (%) of DVT compared with HF-fed WT mice that was concomitant with improved blood flow, reduced neutrophil content and citrullinated histone H3-positive cells (a marker of NETosis) in IVC thrombus. Exogenous Fn-EDA potentiated NETosis in neutrophils stimulated with thrombin-activated platelets via TLR4. Genetic deletion of TLR4 in Fn-EDAfl/fl mice, which constitutively express Fn-EDA, reduced DVT compared with Fn-EDAfl/fl mice. Conclusion: These results demonstrate a previously unknown role of Fn-EDA in the modulation of DVT, which may be an important mechanism promoting DVT in the setting of obesity. Figure Disclosures No relevant conflicts of interest to declare.


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