Recurrent Deep Vein Thrombosis Research Articles

Clinical characteristics of lower extremity deep vein thrombosis in young vs. middle-aged adults: a single-center retrospective study.

The incidence of deep vein thrombosis (DVT) in the lower extremities is increasing in the younger population. However, there are fewer reported comparisons in the literature for lower extremity DVT. Patients aged <40 years admitted with lower-extremity DVT between January 2018 and December 2023 were retrospectively analyzed and followed up for 1 year. A total of 61 patients were included in the study and divided into two groups: 33 patients over 30 years of age (middle-aged group) and 28 patients under 30 years of age (young group). A significant gender difference was observed, with a higher proportion of males in the young group compared to the middle-aged group (P < 0.001). Five patients in the young group were treated with anticoagulation alone, whereas all patients in the middle-aged group underwent endovascular therapy. A higher prevalence of inferior vena cava thrombosis in the young group compared to the middle-aged group (60.71% vs. 33.3%, P = 0.032). The proportion of iliac vein stenosis was significantly higher in the middle-aged groups than in the young group (P = 0.002). There was no statistically significant difference in venous function scores (Villalta and rVCSS) between the two groups during both the preoperative period and the postoperative follow-up (P > 0.05). The incidence of lower-extremity DVT post-thrombotic syndrome and thrombus recurrence was higher in the young group than in the middle-aged group at 1 year postoperatively (PTS: 78.57% vs. 33.3%, P < 0.001, and thrombus recurrence: 28.57% vs. 9.09%, P < 0.05). Univariate and multivariate analyses revealed that inferior vena cava thrombosis was an independent risk factor for severe DVT post-thrombotic syndrome and recurrent DVT (P < 0.05), whereas gender was an independent risk factor for recurrent DVT (P < 0.05). This study suggests differences in the clinical characteristics and prognosis of lower-extremity DVT.

The impact of COVID-19 on the prognosis of deep vein thrombosis following anticoagulation treatment: a two-year single-center retrospective cohort study

BackgroundCoronavirus disease 2019 (COVID-19) has been proved as a significant risk factor for deep vein thrombosis (DVT) after several waves of pandemic. This study aims to further investigate impact of COVID-19 on prognosis of DVT following anticoagulation treatment.MethodsA total of 197 patients with initially detected DVT and meanwhile accomplishing at least 3 months anticoagulation treatment were identified from our hospital between January 2021 and December 2022. DVT characteristics, clinical data, and exposure to COVID-19 were recorded for multivariable logistic regression analysis to identify DVT aggravation related risk factors. Propensity score matching (PSM) was used to balance baseline covariates. Kaplan–Meier curves and Log-Rank test were performed to exhibit distribution of DVT aggravation among different subgroups.ResultsIn 2022, patients exhibited higher incidence rates of DVT aggravation compared to those in 2021 (HR:2.311, P = 0.0018). The exposure to COVID-19, increased red blood cell count, increased D-dimer level and reduced prothrombin time were found to be associated with DVT aggravation (P < 0.0001, P = 0.014, P < 0.001, P = 0.024), with only exposure to COVID-19 showing a significant difference between two years (2022:59/102, 57.84%, 2021:7/88, 7.37%, P < 0.001). In PSM-matched cohorts, the risk for DVT aggravation was 3.182 times higher in COVID-19 group compared to the control group (P < 0.0001). Exposure to COVID-19 increased the risk of DVT aggravation among patients who completed three months anticoagulant therapy (HR: 5.667, P < 0.0001), but did not increase incidence rate among patients who completed more than three months anticoagulant therapy (HR:1.198, P = 0.683). For patients with distal DVT, COVID-19 was associated with a significant increased risk of DVT recurrence (HR:4.203, P < 0.0001). Regarding principal diagnoses, incidence rate of DVT aggravation was significantly higher in COVID-19 group compared to the control group (Advanced lung cancer: P = 0.011, surgical history: P = 0.0365, benign lung diseases: P = 0.0418).ConclusionsOur study reveals an increased risk of DVT aggravation following COVID-19 during anticoagulation treatment, particularly among patients with distal DVT or those who have completed only three months anticoagulant therapy. Adverse effects of COVID-19 on DVT prognosis were observed across various benign and malignant respiratory diseases. Additionally, extended-term anticoagulant therapy was identified as an effective approach to enhance DVT control among patients with COVID-19.

Recurrent Deep Vein Thrombosis (DVT) and Inferior Vena Cava (IVC) Filter: Should a Computed Tomography (CT) Venogram and Inferior Vena Cavagram Be the Standard of Care?

Recurrent Deep Vein Thrombosis (DVT) and Inferior Vena Cava (IVC) Filter: Should a Computed Tomography (CT) Venogram and Inferior Vena Cavagram Be the Standard of Care?

Management of Deep Vein Thrombosis: An Update Based on the Revised AWMF S2k Guideline.

Deep vein thrombosis (DVT) and pulmonary embolism (PE) are the most common manifestations of venous thromboembolism (VTE). Most DVTs affect the lower-extremity veins. Since the symptoms of DVT are non-specific, a prompt and standardised diagnostic work-up is essential to minimise the risk of PE in the acute phase and to prevent thrombosis progression, post-thrombotic syndrome and VTE recurrence in the long-term. Only recently, the AWMF S2k guidelines on Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism have been revised. In the present article, we summarize current evidence and guideline recommendations focusing on lower-extremity DVT (LEDVT). Depending on whether the diagnostic work-up is performed by a specialist in vascular medicine or by a primary care physician, different diagnostic algorithms are presented that combine clinical probability, D-dimer testing and diagnostic imaging. The diagnosis of ipsilateral recurrent DVT poses a particular challenge and is presented in a separate algorithm. Anticoagulant therapy is an essential part of therapy, with current guidelines clearly favouring regimens based on direct oral anticoagulants over the traditional sequential therapy of parenteral anticoagulants and vitamin K antagonists. For most DVTs, a duration of therapeutic-dose anticoagulation of at least 3 to 6 months is considered sufficient, and this raises the question of the risk of VTE recurrence after discontinuation of anticoagulation and the need for secondary prophylaxis in the long-term. Depending on the circumstances and trigger factors that have contributed to the occurrence of DVT, management strategies are presented that allow decision-making taking into account the individual bleeding risk and patient's preferences.

Systemic immune-inflammation index as a novel hematological marker for predicting the recurrence of deep venous thrombosis.

To determine whether there was a possible predictive relationship between systemic immune-inflammation index (SII) and recurrence of deep venous thrombosis (DVT). A total of 231 patients with DVT who met the study criteria and whose data could be accessed were enrolled to this retrospective single-centered cross-sectional study. Of them 26 patients with DVT recurrence consisted of the study group (Group 1) while remaining 205 cases without recurrence were considered as the control population (Group 2). The patients' basic clinical features and laboratory results from the complete blood count (CBC) test were recorded and compared between groups. Following univariate analyses, a multivariate logistic regression analysis was performed to identify the independent predictors of the recurrence of DVT. Additionally, a receiver-operating characteristic (ROC) curve analysis was performed to detect the cut-off values of the predictors with sensitivity and specificity rates. There were no significant differences between the groups for basic clinical features, except for diabetes mellitus, pulmonary embolism, and atrial fibrillation. Although the univariate analysis revealed that the median values of NLR, PLR, and SII were significantly higher in the DVT recurrence group, only SII was determined to be a significant and independent predictor of DVT recurrence in the multivariate logistic regression analysis. According to ROC curve analysis, SII of 1685 × 103/mm3 constituted the cut-off value for predicting DVT recurrence with 61.5% sensitivity and 76.6% specificity (AUC = 0.686, p = .001). The present study demonstrated for the first time in the literature that SII significantly predicted the recurrence of DVT.

Dabigatran etexilate versus warfarin in cerebral venous thrombosis in Chinese patients (CHOICE-CVT): An open-label, randomized controlled trial.

The efficacy and safety of dabigatran etexilate for Chinese patients with cerebral venous thrombosis (CVT) has not been well established. CHOICE-CVT was an exploratory, single-center, randomized, open-label study in the National Center for Neurological Disorders involving Chinese patients with CVT aged 18 to 80 years who were randomly assigned (1:1) to either dabigatran etexilate or warfarin. Oral anticoagulants were initiated after 10-15 days of LMWH. The primary efficacy and safety endpoints included the number of patients with recurrent CVT and/or deep venous thrombosis (DVT) and major clinical bleeding within 180 days. Secondary efficacy endpoints included venous recanalization and change in papilledema at day 180. Secondary safety outcomes comprised death, clinical nonmajor bleeding, and any bleeding. The study was registered with ClinicalTrials.gov under NCT03930940. Between October 2017 and February 2023, a total of 89 patients were enrolled and randomly assigned to receive either dabigatran etexilate (n = 44) or warfarin (n = 45). At day 180, the dabigatran etexilate group showed a statistically nonsignificant but likely clinically significant number of patients with recurrent CVT and/or DVT (8 (18.2%; 95% CI, 6.3-30.0) vs 3 (6.7%; 95% CI, 0.0-14.2), p = 0.099, with a power (1-β) of 38.401%) compared with the warfarin group. The dabigatran etexilate group showed a comparable number of patients with clinical major bleeding (0 (0) vs 0 (0) p = 1.000), and clinical nonmajor bleeding (1 (2.3%; 95% CI, 0.0-6.9) vs 1 (2.2%; 95% CI, 0.0-6.7)) but demonstrated a lower risk of any bleeding (1 (2.3%; 95% CI, 0.0-6.9) vs 9 (20.0%; 95% CI, 7.8-32.2)) compared with the warfarin group. Most patients in both groups achieved venous recanalization according to the Modified Qureshi scale (27 (75%; 95% CI, 60.1-89.9) in the dabigatran etexilate group vs 34 (82.9%; 95% CI, 70.9-95.0) in the warfarin group) and exhibited improvement in papilledema as per the Frisén classification (35 (97.2%; 95% CI, 91.6-100.0) in the dabigatran etexilate group vs 37 (88.1%, 95% CI, 77.9-98.3) in the warfarin group). These findings regarding efficacy and safety support the consideration of dabigatran etexilate therapy as a viable treatment option for Chinese patients with CVT.

Congenital Hypodysfibrinogenemia due to γ326Cys→Tyr Mutation: Third Ever-Described Case Associated with Recurrent Venous Thrombosis and COVID Vaccine

&lt;b&gt;&lt;i&gt;Introduction:&lt;/i&gt;&lt;/b&gt; Congenital fibrinogen disorders are a heterogenous group of fibrinogen defects. &lt;b&gt;&lt;i&gt;Case Presentation:&lt;/i&gt;&lt;/b&gt; Here, we describe hypodysfibrinogenemia in a 33-year-old female patient with provoked recurrent deep vein thrombosis (DVT) diagnosed based on decreased functional and antigenic fibrinogen levels with a decreased functional/antigenic fibrinogen ratio. Definitive diagnosis of congenital hypodysfibrinogenemia is done by genotyping using whole-exome sequencing, which identified the γ326Cys→Tyr mutation combined with single-nucleotide polymorphisms: rs2070011 and rs2070018 in FGA and rs1049636 in FGG. Fibrin structure assays showed reduced maximum polymerization rate. The mother of the proband shares the same γ326Cys→Tyr mutation and experienced a provoked DVT. &lt;b&gt;&lt;i&gt;Conclusion:&lt;/i&gt;&lt;/b&gt; Our case with DVT is the third ever-described occurrence of the mutation γ326Cys→Tyr that is associated with hypodysfibrinogenemia. The mechanism by which this mutation induces thrombosis remains unknown. Due to the high recurrence risk of thrombosis, the patient was treated with long-term reduced dose of rivaroxaban (10 mg daily) as secondary prophylaxis.

Deep venous thrombosis in autosomal dominant polycystic kidney

ABSTRACT Autosomal dominant polycystic kidney disease (ADPKD) is a prevalent genetic cystic condition that results in progressive loss of renal function. The disease is associated with various complications, including compression of adjacent structures like the inferior vena cava (IVC), which can lead to deep vein thrombosis (DVT) and ultimately, pulmonary embolism. This report details a case of a 41-year-old patient with ADPKD who presented with left leg DVT. Imaging revealed IVC compression due to enlarged kidneys, and blood tests indicated low levels of coagulation proteins C and S. The patient is hypertensive and has advanced renal failure (glomerular filtration rate [GFR] 12.6 mL/min) but has not yet initiated dialysis. The patient was initiated on treatment with the novel oral anticoagulant, apixaban, and exhibited favorable outcomes. His DVT is believed to be the result of multiple factors acting in concert. IVC compression, a significant precipitant doctor of DVT, is frequently observed in ADPKD patients, with rates as high as 15% in severe cases and 15% in mild cases, according to a study by Yin et al. The incidence of venous thromboembolism (VTE) in patients with chronic kidney disease increases as GFR decreases, with rates 2.5–5.5-fold higher in moderate-to-severe end-stage renal disease than in the general population. VTE is a multifactorial disease with both acquired and inherited thrombophilia factors. This particular case presents a unique event within our medical practice, as it is relatively uncommon to witness such a correlation between DVT and polycystic kidney disease. It serves as an exemplification of a case that necessitates diligent monitoring due to the heightened risk of recurrent DVT.

Cost-Effectiveness of Performing Reference Ultrasonography in Patients with Deep Vein Thrombosis.

The diagnosis of recurrent ipsilateral deep vein thrombosis (DVT) with compression ultrasonography (CUS) may be hindered by residual intravascular obstruction after previous DVT. A reference CUS, an additional ultrasound performed at anticoagulant discontinuation, may improve the diagnostic work-up of suspected recurrent ipsilateral DVT by providing baseline images for future comparison. To evaluate the cost-effectiveness of routinely performing reference CUS in DVT patients. Patient-level data (n = 96) from a prospective management study (Theia study; NCT02262052) and claims data were used in a decision analytic model to compare 12 scenarios for diagnostic management of suspected recurrent ipsilateral DVT. Estimated health care costs and mortality due to misdiagnosis, recurrent venous thromboembolism, and bleeding during the first year of follow-up after presentation with suspected recurrence were compared. All six scenarios including reference CUS had higher estimated 1-year costs (€1,763-€1,913) than the six without reference CUS (€1,192-€1,474). Costs were higher because reference CUS results often remained unused, as 20% of patients (according to claims data) would return with suspected recurrent DVT. Estimated mortality was comparable in scenarios with (14.8-17.9 per 10,000 patients) and without reference CUS (14.0-18.5 per 10,000). None of the four potentially most desirable scenarios included reference CUS. One-year health care costs of diagnostic strategies for suspected recurrent ipsilateral DVT including reference CUS are higher compared to strategies without reference CUS, without mortality benefit. These results can inform policy-makers regarding use of health care resources during follow-up after DVT. From a cost-effectiveness perspective, the findings do not support the routine application of reference CUS.

Non-Vitamin K Antagonist Oral Anticoagulants versus Low Molecular Weight Heparin for Cancer-Related Venous Thromboembolic Events: Individual Patient Data Meta-Analysis.

Venous thromboembolism (VTE) is a leading cause of morbidity and mortality in cancer patients. Low molecular weight heparin (LMWH) has been the standard of care but new guidelines have approved the use of non-vitamin K antagonist oral anticoagulants (NOAC). By conducting an individual patient data (IPD) meta-analysis of randomised controlled trials (RCTs) comparing the outcomes of NOAC versus LMWH in cancer patients, we aim to determine an ideal strategy for the prophylaxis of VTE and prevention of VTE recurrence. Three databases were searched from inception until 19 October 2022. IPD was reconstructed from Kaplan-Meier curves. Shared frailty, stratified Cox and Royston-Parmar models were fit to compare the outcomes of venous thromboembolism recurrence and major bleeding. For studies without Kaplan-Meier curves, aggregate data meta-analysis was conducted using random-effects models. Eleven RCTs involving 4844 patients were included. Aggregate data meta-analysis showed that administering NOACs led to a significantly lower risk of recurrent VTE (RR = 0.65; 95%CI: 0.50-0.84) and deep vein thrombosis (DVT) (RR = 0.60; 95%CI: 0.40-0.90). In the IPD meta-analysis, NOAC when compared with LMWH has an HR of 0.65 (95%CI: 0.49-0.86) for VTE recurrence. Stratified Cox and Royston-Parmar models demonstrated similar results. In reducing risks of recurrent VTE and DVT among cancer patients, NOACs are superior to LMWHs without increased major bleeding.

May Thurner Syndrome: Diagnostic and Therapeutic Outcomes in an Institutional Setting

Introduction: May-Thurner syndrome (MTS), a chronic compression of left common iliac vein (CIV) against the lumbar vertebrae by overlying common iliac artery is an underappreciated contributor to left leg deep venous thrombosis. Even though the estimated prevalence of MTS is high in left-leg deep vein thrombosis (DVT) cases per autopsy studies, the clinical association remains low. Since the screening for MTS in patients with recurrent left-leg DVTs is not a common practice, diagnosis can either be incidental or a combination of clinical presentation and objective testing. Diagnosis is challenging via ultrasound (US) due to the deep anatomical location of iliac veins; therefore, invasive imaging including Contrast venography (CTV) is needed for confirmation. The management of MTS has been reformed over the years with the advances in minimally invasive therapeutic tools. We sought to review the management and outcomes of the MTS adult population by conducting a retrospective review to identify all adult cases of MTS at our academic safety net hospital between 2010 and 2022. Methods: Data was extracted and analyzed for baseline parameters including age, gender, ethnicity, and body mass index (BMI). Presenting symptoms, imaging used for MTS diagnosis (including US Doppler, magnetic resonance venography or CTV), and interventional approaches (catheter or systemic thrombolysis, thrombectomy, balloon angioplasty and/or stent placement) were additionally documented. SAS v9.4 (Cary, NC) was used for statistical analysis. Results: A total of 19 cases, predominantly females 68% (13/19), with a median age of 48 years and a mean BMI of 28.84 kg/m 2 diagnosed with MTS were identified. The predominant initial presentation was left lower extremity edema and pain 36% (7/19) later found to have a DVT, followed by non-healing venous ulcer 15% (3/19) and pelvic congestion syndrome 0.05 % (1/19). Whereas 42% (8/19) of the patients were diagnosed with MTS while undergoing evaluation for chronic and recurrent DVTs. All patients were diagnosed following CTV using transvenous pressures with the formation of collateral veins and a pressure gradient of more than 2 mm Hg across iliofemoral stenosis at rest. Sixteen patients (84%) underwent endovascular stent placement, while one patient underwent Balloon Angioplasty only. One patient refused any intervention for MTS however underwent great saphenous vein ablation for chronic venous wounds. In view of anticoagulation therapy, the initial choice in the most cases was low molecular weight heparin (LMWH), which was later transitioned to other choices, 81 % (10/16) were switched to direct oral anticoagulants, (apixaban= 10, rivaroxaban=3) due to ease of administration and compliance, while 19 % (3/16) were continued on LMWH for patient preference and pregnancy. Post-intervention complications consisted of stent failure in 25% (4/16) patients; related to stent thrombosis and Post-thrombotic Syndrome (PTS) in 12.5 % (2/16) patients. No bleeding complications were seen post any case of endovascular stent placement. Conclusion: This review was a highly educational experience giving us insight into this unacknowledged syndrome. Multiple factors could be contributing to this underdiagnosis, including invasive gold standard imaging for MTS, more easily recognizable risk factors for DVTs that can overshadow MTS, and a smaller number of data and studies. Some authorities have advocated a need to rule out MTS in young women with acute left lower extremity DVT, a clear female predominance was also noted (68%) in our analysis. Post-stent placement, systemic anticoagulation for 3-6 months is recommended, in our review, all patients (100%) who underwent stent placement were started on anticoagulation for at least 3 months. A small sample size, patients lost to follow-up (poor outcomes and complications could have been missed) and a single institutional review were some limitations of our study, however with a rare disease such as MTS with 2-3% prevalence it is difficult to conduct a large size, multi- institutional analysis. This study contributes to the limited literature available on MTS.

Recurrent Leg Deep Vein Thrombosis in a Female with Atypical Right-Sided Variant of May Thurner Syndrome and Factor V Leiden Mutation

I ntroduction: May-Thurner syndrome (MTS) is a condition in which the right common iliac artery presses on the left common iliac vein, causing it to narrow over time. Right-sided MTS is a rare event. We present this unique case of recurrent right leg deep vein thrombosis in a female with atypical right sided variant of MTS and Factor V Leiden mutation. Case Presentation: A 34-year-old lady with with no signficiant past medical history presented to the hospital with right lower leg edema, tingling, and discolouration. She was taking oral contraceptive pills. Vitals were unremarkable. Doppler studies revealed right lower extremity deep vein thrombosis in the right external iliac vein, common iliac vein and common femoral vein. She was started on an intravenous heparin drip and subsequently had a thrombectomy procedure. She was subsequently discharged home on apixaban anticoagulation. We opted to request a hypercoagulable and vasculitis workup in light of the extensive thrombosis, despite the OCP risk factor, and discovered a heterozygous Factor V Leiden mutation. She returned to the hospital 2 days later with worsening right leg pain. Repeat Doppler studies demonstrated acute right femoral and popliteal deep vein thrombosis. CT angiography of abdomen and pelvis revealed significant right femoral vein DVT that extends into the External iliac vein, common iliac vein, and IVC to at least slightly below renal vein level. Invasive Radiology was consulted and she had a venogram and intravascular ultrasound (IVUS) which showed high-grade right common iliac vein and IVC stenosis (&amp;gt;90%) at L4 superior endplate. Iliac stenting and angioplasty improved iliac and inferior IVC diameters. Patient also underwent thrombectomy &amp; IVC filter placement. The diagnostic venography revealed a popliteal-to-proximal femoral vein rethrombosis. This was treated with tissue plasminogen activator (TPA) by 8 French power pulse AngioJet and a mechanical thrombectomy. This resulted in partial clearing of the common femoral vein with persistent narrowing and thrombosis of the popliteal vein. TPA was infused at 1 mg/hour with a 5 French McNamara lysis catheter over the popliteal region.Patient transferred to ICU for overnight intravenous thrombolysis. The patient was then re-initiated on the heparin drip and then bridged to therapeutic warfarin. Discussion: MTS is a phenomenon commonly described as an acquired stenosis of the left common iliac vein secondary to compression by the right common iliac artery 1. It has been associated with genetic clotting factor abnormalities, repeated pregnancies, postpartum, contraceptive pills, prolonged immobilization, scoliosis, anti-phospholipid syndrome, COVID-19, and recurrent DVT 2. In patients with left lower-limb DVT, the incidence of MTS is about 20-50%. Right lower limb DVT in patients with right sided variant of MTS is rare. Right lower limb DVT with right sided variant of MTS with co-existing Factor V Leiden mutation is even rarer. Ultrasound imaging typically cannot detect the high pelvic abnormalities seen in MTS. Contrast venography, MRI, or IVUS are needed to diagnose MTS 4. As was the case in our patient, venography should be done to evaluate stenosis and iliac vein compression hemodynamics after thrombus removal. Treatment of MTS depends on the presentation, severity of symptoms, and whether the patient has developed DVT. Conservative treatment such as compression stockings can be used for patients who are asymptomatic or have mild symptoms. For patients with moderate symptoms or DVT, endovascular treatment with stenting is recommended 5. Conclusion: This is a very atypical presentation of MTS variation affecting the right lower extremity with limited description in the literature. To our knowledge, this is the first case presentation of right sided MTS resulting in a DVT in a patient with factor V Leiden mutation. MTS is a potentially treatable and often-overlooked pathology. In the era of expanded endovascular management of MTS, recognition of this disease entity is essential for early diagnosis and treatment.

SH2B3 Mutations in Patients with JAK2-Negative Erythrocytosis

Introduction: Erythrocytosis is a common reason for referral to Hematology clinics. Molecular testing for JAK2 mutations, often by next-generation sequencing (NGS), has become part of the diagnostic workup of patients with erythrocytosis, increasing the molecular characterization of this previously undifferentiated patient population. A recent study (Bhai et al. 2022) found that 4% of patients referred for JAK2-negative erythrocytosis harboured variants in the SH2B3 gene, which encodes the LNK protein, a known negative regulator of JAK2/STAT5 signaling. The objective of this study was to characterize a cohort of patients with JAK2-negative erythrocytosis with SH2B3 mutations, evaluating clinical presentation, hematologic parameters, as well as cardiovascular and thrombotic outcomes. Methods: All adult patients referred to the London Health Sciences Centre, a tertiary care centre in Southwestern Ontario, for assessment of erythrocytosis (hemoglobin &amp;gt;160g/L in women; &amp;gt;165g/L in men) between January 1, 2018 and December 31, 2022 and who underwent NGS testing were reviewed. NGS testing was performed using the Oncomine Myeloid Research Assay (Thermofisher Scientific, MA, USA). Patients with JAK2, CALR, MPL, or BCR::ABL1 mutations were excluded. Patients without JAK2 mutations ( JAK2-negative) with variants in SH2B3 were further characterized. Clinical variables included risk factors for secondary erythrocytosis and occurrence of arterial/venous thrombotic events. Laboratory variables included complete blood count and white cell differential. Results: Sixteen patients with JAK2-negative erythrocytosis met inclusion criteria with mutations in SH2B3. All mutations in SH2B3 were characterized as Tier III variants (Variants of Uncertain Significance). Laboratory and clinical parameters in this patient population are presented in Table 1. Mean hemoglobin was 181 g/L, hematocrit 0.54 L/L, leukocyte count 7.2 x10 9 cells/L, and platelets 229 x10 9 cells/L. Secondary causes of erythrocytosis were identified in 13 (81%) patients: 10 (63%) reported active smoking or had a smoking history, 4 (25%) had obstructive sleep apnea, 2 (13%) had chronic obstructive pulmonary disease, 1 (6%) had a right-to-left cardiac shunt, 4 (25%) were on an SGLT2 inhibitor, and 1 (6%) reported testosterone use. Of note, one patient had a history of stroke and another had a myocardial infarction both of which predated the development of erythrocytosis and NGS testing. One patient had a recurrent deep vein thrombosis (DVT) at the time of NGS testing. Full molecular data and comparative analysis with other cases of JAK2-negative erythrocytosis will be presented at ASH. Discussion: SH2B3 mutations have been identified in rare cases of congenital erythrocytosis and implicated in the pathogenesis of erythropoiesis. To our knowledge, this is the largest cohort of patients with JAK2-negative erythrocytosis with variants in SH2B3 reported to date. This cohort showed similar laboratory parameters and prevalence of secondary risk factors for erythrocytosis as previously reported in the general population of patients with JAK2-negative erythrocytosis at our centre (Chin-Yee et al. 2022). Thrombotic outcomes were also similar, and lower than patients with polycythemia vera. Given the high prevalence of potential secondary causes of erythrocytosis in our cohort we cannot confirm that SH2B3 mutations are causally implicated in elevated hemoglobin in these cases. Nonetheless, we cannot exclude that SH2B3 variants may confer an additional risk factor for erythrocytosis in this patient population. Further research is required to elucidate the potential impact of SH2B3 variants in patients with JAK2-negative erythrocytosis.

May-Thurner Syndrome Should be Explored As a Possible Existing Risk Factor in Pediatric Patients Presenting with Non-Central Venous Line Associated Deep Vein Thrombosis

Background: The compression of the left common Iliac vein (LCIV) by the right common iliac artery is called May-Thurner Syndrome (MTS). The incidence of this anatomic variance is unknown, though it is estimated to be 18-49%. It is currently established that the chronic compression of the iliac vein outflow results in various clinical symptoms including edema, pain, discoloration, skin ulcers and venous thromboembolism. Deep vein thrombosis (DVT) of the LCIV is the most reported presentation of MTS in pediatrics. DVTs in pediatric patients are rare and often provoked by a central venous line (CVL). Patients without a CVL in the context of left lower extremity (LLE) DVT often do not undergo MTS work-up if the DVT does not include the LCIV. To assess if MTS is a risk factor for LLE DVT distally to the LCIV, we describe an institutional incidence of MTS in two pediatric patient populations: those with non CVL-associated LLE DVTs and a control group composed of pediatric patients undergoing imaging that includes the LLE vasculature for unrelated reasons. Methods: This was a single center retrospective study. We identified patients with a non CVL-associated LLE DVT seen at our institution using ICD-10 codes as well as a cohort of patients who received imaging of the LLE vasculature for reasons other than DVT using radiology MRI protocol codes.Electronic medical records were reviewed for demographic variables such as age and gender. Patients without DVTs had imaging reviewed for the incidental finding of MTS. Those with DVTs had information collected regarding clinical characteristics such as coagulation panels, thrombosis risk factors (thrombophilic states, oral contraceptive use, obesity), and therapy received. All images were reviewed by a pediatric radiologist. Results: A total of 35 patients with non CVL-associated LLE DVTs, and 752 patients without CVL and without LLE DVTs were identified. Of those with LLE DVTs, 19 (54%) were found to have MTS. Of these 19 patients, 5 (26%) were obese, 14 (74%) were on oral contraceptive pills, and 8 (42%) reported prothrombotic risks (7 heterozygous for Factor V Leiden, 1 heterozygous for protein C deficiency). Two patients (10%) had no additional risk factors for thrombosis. Of those without LLE DVTs, 6 patients (0.01%) were incidentally diagnosed with MTS. Interestingly we also described one patient with non CVL-associated LLE DVT distally to the LCIV with MTS. Conclusions: In our study, the presence of MTS was significantly higher in the non CVL-associated LLE DVT group than in the control group. This study suggests that MTS should be considered, and associated imaging performed, in pediatric patients with a non CVL-associated LLE DVT, considering the incidence revealed by this single institutional case series. The presence of MTS in pediatric patients may significantly impact the life-long risk of DVT recurrence. Given that aging adversely impacts lower limb venous return (exacerbated by central obesity, sedentary lifestyle, and other risk factors) and that pregnancy is a known trigger of DVT in patients with MTS, knowledge of prior MTS may impact patient care over the lifespan.

Should we add antiplatelet agents to current deep venous thrombosis treatments? A Cochrane Review summary with commentary.

Venous thromboembolism (VTE) is an important complication in rehabilitation practice despite preventive measures. The management can be complicated because patients may have co-existing cardiovascular comorbidities. To assess the effects of antiplatelet agents in addition to current best medical practice (BMP) compared to current BMP (with or without placebo) for the treatment of deep venous thrombosis (DVT). A summary of the Cochrane Review by Flumignan et al. (2022), with comments from a rehabilitation perspective. The review included six studies with 1625 eligible participants, with data up to 37.2 months of follow-up. When used after standard initial treatment with anticoagulants, antiplatelet agents such as aspirin in addition to BMP, may reduce recurrence of DVT or pulmonary embolism, when compared to BMP plus placebo in a chronic DVT setting and there may be a lower risk for post-thrombotic syndrome in patients with acute DVT. There is no clear difference in side effects, major bleeding, or pulmonary embolism (PE) with the use of antiplatelet agents. Adding antiplatelet agents to standard anticoagulation treatment in patients with VTE could provide benefit without increasing risks in selected patient groups. However, high quality studies with a long-term follow up are needed, including patients in rehabilitation settings.

Single-center experience with the ClotTriever BOLD catheter for deep vein thrombosis percutaneous mechanical thrombectomy of the lower extremity.

The ClotTriever System is a percutaneous mechanical thrombectomy system used to treat deep vein thrombosis (DVT). The BOLD catheter is a newer compatible component with a modified coring element for which reported outcomes are limited. This retrospective study aims to assess the preliminary procedural safety and success data for patients treated with the BOLD catheter. All consecutive patients with symptomatic lower extremity DVT who underwent thrombectomy with the BOLD catheter between 23 November 2021 and 26 June 2022 at a single center were included. Baseline and procedural characteristics were reported. The primary outcome, intraprocedural safety, was assessed by a chart review of recorded intraprocedural adverse events (AEs) or device malfunction. The secondary outcome, procedural success, was defined as ≥75% reduction in the total occlusion across treated venous segments. This was assessed by an interventionalist review of pre- and postprocedural venograms. Additional outcomes included length of postprocedural hospital stay and assessment of AEs at discharge and a 30-day follow-up visit. Eleven patient cases were reviewed. The median patient age was 65 years, the majority were women, and all were treated unilaterally. All procedures were completed in a single session without intraprocedural AEs or device malfunction. The median procedural blood loss was 50 ml. A review of pre- and postprocedural venograms showed that 35 venous segments were treated, including the femoral (n = 9), common femoral (n = 9), external iliac (n = 10), and common iliac (n = 7) veins. Procedural success was achieved in 10 patients (90.9%), and the median reduction rate in venous occlusion was 100%. The median length of postprocedural hospital stay was 1 day, and no AEs were noted at discharge (N = 11). One adverse event occurred among the eight patients who completed their follow-up visit. A patient with advanced-stage cancer and medication failure had a recurrent DVT 13 days postprocedure, which was not related to the device or procedure. No safety concerns concerning the BOLD catheter were raised during the review of the cases included in this analysis, and the device was successful in reducing venous occlusion in patients with symptomatic proximal lower extremity DVT.

Management of Recurrent Venous Thromboembolism in Severe Immune Thrombocytopenia: A Case Report and a Review of the Literature.

We report a case of a 58-year-old man with recurrent unprovoked deep vein thrombosis (DVT) and severe immune thrombocytopenia (ITP) with a platelet count of 19 × 109/L. We further review studies reporting venous thromboembolism (VTE) in patients with severe ITP (≤ 35 × 109/L) and identified 14 patients highlighting VTE risk factors and management of these patients. The present case had several risk factors for VTE (previous DVT, obesity, heterozygosity for factor V Leiden mutation, and previous splenectomy). The patient was initially treated with low-molecular-weight heparin followed by long-term apixaban treatment. The literature review together with our case demonstrates that VTE in severe ITP (≤ 35 × 109/L) can occur in patients with VTE risk factors and antithrombotic management of these patients can be achieved without bleeding depending on severity of thrombocytopenia either by full or reduced dose of anticoagulation together with ITP therapy.

Protein C Deficiency with Recurrent Deep Vein Thrombosis Complicated with Post Thrombotic Syndrome- A Case Report and Mini Review

Protein C is a vitamin K dependent glycoprotein produced by the liver.  Protein C acts as a naturally occurring anticoagulant and its deficiency, predisposes the individual to a state of thrombosis, particularly venous thromboembolism. Post-thrombotic syndrome (PTS) is a chronic venous insufficiency manifestation following an episode of deep-vein thrombosis (DVT). We presented a case of 56 year old Malay man who was diagnosed with Protein C deficiency and presented with recurrent DVT complicated with PTS.

Coexistence of Systemic Lupus and Antiphospholipid Syndrome in a Male Patient: Atypical Case Presentation

Abstract Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) are two distinct autoimmune disorders characterized by dysregulated immune responses, organ involvement, and the presence of autoantibodies in the bloodstream. Primary Antiphospholipid Syndrome (APS) can occur among individuals who are generally healthy and have no history of an underlying systemic autoimmune illness. In contrast, several other systemic autoimmune diseases, including SLE, have the potential to cause secondary Antiphospholipid Syndrome (APS). While Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) affect women of childbearing age, there are only a few reported case studies documenting the presence of SLE or APS individually in male patients. However, the coexistence of both conditions in a male patient is extremely rare, with only a few documented cases reports available to date. This case report presents a unique occurrence of concurrent SLE and APS in a forty-year-old male patient, highlighting the rarity of such a presentation. The patient presented with per rectal and mucosal bleeding and had been on warfarin therapy for 16 years due to a history of recurrent Deep Vein Thrombosis (DVT), but was not evaluated further. Immune-related laboratory results revealed positive antinuclear antibodies, anti-dsDNA antibodies, anticardiolipin antibodies, lupus anticoagulants, and direct Coombs test. Concurrent SLE and APS were diagnosed by meeting the classification criteria for both diseases. This report adds to the medical literature and emphasizes the significance of considering concurrent SLE and APS as a potential diagnosis in males. Further research is needed to enhance our understanding of the underlying mechanisms and the best approaches for managing this rare coexistence.

FRI229 Bilateral Adrenal Hemorrhage Following Deep Tissue Body Massage

Abstract Disclosure: M. Antony: None. S. Gundlapally: None. P. Russell: None. S. Storm: None. S. Patel: None. V. Verma: None. R. Kant: None. Introduction: Adrenal hemorrhages have been reported in patients undergoing ACTH stimulation test using bovine- source ACTH. It can also be seen in severe stressful situations such as septicemia and extensive thermal burns due to sudden and profound adrenocortical stimulation by endogenous ACTH. Idiopathic spontaneous adrenal hemorrhage and motor vehicle accident induced adrenal hemorrhage have also been rarely reported. Coagulopathy, thromboembolic disease, and postoperative state are 3 major risk factors associated with bilateral adrenal hemorrhage. We present a unique case of a patient who presented with bilateral adrenal hemorrhage following deep tissue body massage. Clinical Case: A 44-year-old male with a past medical history of recurrent pulmonary embolism (PE) and deep venous thrombosis (DVT) due to lupus anticoagulant disorder status-post inferior vena cava (IVC) filter and recent change from Coumadin to Xarelto presented to the ED with a chief complaint of right upper quadrant (RUQ) pain extending to the right flank. Patient experienced unrelenting pain after a deep-tissue massage one week prior to presentation. Patient was seen two days prior to admission for similar complaints, but was discharged after reassuring CT abdomen/pelvis, ultrasound, and labs. He returned after pain worsened, and a repeat CT showed right adrenal hemorrhage and enlargement. Patient denied all other symptoms except pain. Vitals included BP of 144/84 mmhg, RR 20/min, HR 89/min, temp of 98.1°F, and SpO2 of 99%. Physical exam was notable only for RUQ pain and right CVA tenderness. Initial labs showed normal CBC including HGB 13.6g/dL (13-17 g/dL), HCT 37.5% (38.5-52%), and PLT 181K/microL (150-450 K/microL). Glucose 120mg/dL (70-99mg/dL), Na 129mmol/L (135-146 mmol/L), K 3.3mmol/L (3.5-5.2 mmol/L), Cl 87mmol/L (98-107 mmol/L), anion gap 17mmol/L (4-15mmol/L). LFT, serum lipase and CPK were non-contributory. PT was elevated at 24.7s (10-13s) and INR was 2.1 (0.9-1.2). Anticoagulation was stopped for three to four weeks, and nonsurgical management was recommended. Potassium supplementation was given, and the patient was continued on his hypertension medications. On further investigation, origin of the hemorrhage was determined to be traumatic secondary to the massage during which the masseuse walked on the patient. The patient was discharged after three days but was readmitted after continued abdominal pain and hyponatremia. He was diagnosed with a left-sided adrenal hemorrhage on repeat CT scan twelve days after identification of the first. Medical management was successful. Conclusion: Adrenal hemorrhage is a potentially life-threatening condition often caused by trauma, severe burns, or septicemia. Anticoagulant therapy and thromboembolic disease, as seen in our patient, are also risk factors. The atypical trauma induced by the patient's massage likely initiated the hemorrhagic event. Presentation: Friday, June 16, 2023