Shayna Sarosiek

Indications, complications, and management of inferior vena cava filters: the experience in 952 patients at an academic hospital with a level I trauma center.

IMPORTANCE Retrievable inferior vena cava (IVC) filters were designed to provide temporary protection from pulmonary embolism, sparing patients from long-term complications of permanent filters. However, many retrievable IVC filters are left in place indefinitely. OBJECTIVES To review the medical records of patients with IVC filters to determine patient demographics and date of and indication for IVC filter placement, as well as complications, follow-up data, date of IVC filter retrieval, and use of anticoagulant therapy. DESIGN AND SETTING A retrospective review of IVC filter use between August 1, 2003, and February 28, 2011, was conducted at Boston Medical Center, a tertiary referral center with the largest trauma center in New England. PARTICIPANTS In total, 978 patients. Twenty six patients were excluded from the study because of incomplete medical records. INTERVENTION Placement of retrievable IVC filter. MAIN OUTCOME MEASURES In total, 952 medical records were included in the analysis. RESULTS Of 679 retrievable IVC filters that were placed, 58 (8.5%) were successfully removed. Unsuccessful retrieval attempts were made in 13 patients (18.3% of attempts). Seventy-four venous thrombotic events (7.8% of 952 patients included in the study) occurred after IVC filter placement, including 25 pulmonary emboli, all of which occurred with the IVC filter in place. Forty-eight percent of venous thrombotic events were in patients without venous thromboembolism at the time of IVC filter placement, and 89.4% occurred in patients not receiving anticoagulants. Many IVC filters placed after trauma were inserted when the highest bleeding risk had subsided, and anticoagulant therapy may have been appropriate. While many of these filters were placed because of a perceived contraindication to anticoagulants, 237 patients (24.9%) were discharged on a regimen of anticoagulant therapy. CONCLUSION AND RELEVANCE Our research suggests that the use of IVC filters for prophylaxis and treatment of venous thrombotic events, combined with a low retrieval rate and inconsistent use of anticoagulant therapy, results in suboptimal outcomes due to high rates of venous thromboembolism.

Association Between Inferior Vena Cava Filter Insertion in Trauma Patients and In-Hospital and Overall Mortality

Importance Trauma patients admitted to the hospital are at increased risk of bleeding and thrombosis. The use of inferior vena cava (IVC) filters in this population has been increasing, despite a lack of high-quality evidence to demonstrate their efficacy. Objective To determine if IVC filter insertion in trauma patients affects overall mortality. Design, Setting, and Participants This retrospective cohort study used stratified 3:1 propensity matching to select a control population similar to patients who underwent IVC filter insertion at Boston Medical Center (a level I trauma center at Boston University School of Medicine) between August 1, 2003, and December 31, 2012. Among patients with an IVC filter and matched controls, age, sex, race/ethnicity, and Injury Severity Score were entered into a multivariable logistic regression model to calculate a propensity score. Matching was stratified by the date of injury. Main Outcomes and Measures Multivariable logistic regression was used to compare hospital mortality across both groups, adjusting for age, sex, race/ethnicity, Injury Severity Score, and brain injury severity using the head and neck Abbreviated Injury Score. To determine any significant difference in mortality, patient characteristics and mortality data from the National Death Index were analyzed in all patients and in those who survived 24, 48, and 72 hours after injury, as well as at hospital discharge. Results Among 451 trauma patients with an IVC filter and 1343 matched controls without an IVC filter, the mean (SD) age was 47.4 (21.5) years. The median Injury Severity Score overall was 24 (range, 1-75). Based on a mean follow-up of 3.8 years (range, 0-9.4 years), there was no significant difference in overall mortality or cause of mortality in patients with vs without an IVC filter who survived more than 24 hours from the time of injury, independent of the presence or absence of deep vein thrombosis or pulmonary embolism at the time of IVC filter placement. Additional analyses at shorter intervals of 6 months and 1 year after discharge also showed no significant difference between the 2 groups of patients. Eight percent (38 of 451) of the IVC filters were removed at Boston Medical Center during the follow-up period. Conclusions and Relevance The research herein demonstrates no significant difference in survival in trauma patients with vs without placement of an IVC filter, whether in the presence or absence of venous thrombosis. The use of IVC filters in this population should be reexamined because filter removal rates are low and there is increased risk of morbidity in patients with filters that remain in place.

Hematologic relapse in AL amyloidosis after high-dose melphalan and stem cell transplantation

To the editor: Light-chain (AL) amyloidosis is a rare disease in which an underlying clonal plasma cell population generates aberrant immunoglobulin light chains that misfold and form amyloid fibrils, which are deposited in extracellular tissues and organs, resulting in impairment of vital organ

A solitary mediastinal mass due to localized AL amyloidosis: case report and review of the literature

Abstract AL amyloidosis presenting as a solitary mediastinal mass is a rare occurrence, with only a few cases reported in the literature. We describe a case of a man presenting with a mediastinal mass diagnosed as amyloidosis, confirmed by mass spectrometry to consist of lambda light chains. Here we review the literature and discuss treatment options for this rare entity.

High-Dose Melphalan and Stem Cell Transplantation in Patients on Dialysis Due to Immunoglobulin Light-Chain Amyloidosis and Monoclonal Immunoglobulin Deposition Disease

The kidney is the most common organ affected by immunoglobulin light-chain (AL) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD), often leading to end-stage renal disease (ESRD). High-dose melphalan and stem cell transplantation (HDM/SCT) is effective for selected patients with AL amyloidosis, with high rates of complete hematologic response and potential for improved organ dysfunction. Data on tolerability and response to HDM/SCT in patients with ESRD due to AL amyloidosis and MIDD are limited. We analyzed data on toxicity, efficacy, and hematologic and renal response of HDM/SCT in 32 patients with AL amyloidosis and 4 patients with MIDD who were dialysis-dependent for ESRD treated at Boston Medical Center between 1994 and 2016. The most common grade 3/4 nonhematologic toxicities were infections (75%), metabolic abnormalities (56%), mucositis (42%), constitutional symptoms (39%), pulmonary complications (39%), and diarrhea (28%). Treatment related mortality (defined as death within 100 days of SCT) occurred in 8% (3 of 36). A complete hematologic response was achieved in 70% of evaluable patients (19 of 27) at 1 year after HDM/SCT. In the entire cohort, median overall survival (OS) after HDM/SCT was 5.8 years; median OS was 1 year for those who did not achieve a complete hematologic response and 8 years for those who did achieve a complete hematologic response. Twelve patients (33%) underwent kidney transplantation after successful treatment with HDM/SCT at a median of 2.4 years after SCT. HDM/SCT is safe and effective in inducing hematologic complete responses and prolonging survival in patients with ESRD from AL amyloidosis and MIDD. Achievement of a durable hematologic response can make these patients possible candidates for renal transplantation.

Modified High-Dose Melphalan and Autologous Stem Cell Transplantation for Immunoglobulin Light Chain Amyloidosis

High-dose melphalan and autologous stem cell transplantation (HDM/SCT) have been used in patients with immunoglobulin light chain (AL) amyloidosis for over 2 decades now with durable responses, prolonged survival, and decreasing treatment-related mortality. Historically, patients with poorer baseline functional status, advanced age, renal compromise, and cardiac involvement have been treated with a risk-adapted modified conditioning dose of melphalan (mHDM) of 100 to 140 mg/m2 before SCT. In part because of these baseline characteristics, patients receiving mHDM/SCT have had poorer outcomes compared with patients receiving full-dose melphalan at 200 mg/m2. With the advent of novel therapeutic agents such as proteasome inhibitors, immunomodulatory agents, and monoclonal antibodies for the treatment of AL amyloidosis, it is imperative to understand the long-term effects of mHDM/SCT. Here we report the long-term outcomes of 334 patients with AL amyloidosis treated with mHDM/SCT. Median overall survival was 6.1 years and median event-free survival 4.3 years, with median overall survival reaching 13.4 years for patients who had achieved a hematologic complete response (CR). Overall hematologic response rate was 69%, and treatment-related mortality was 3% after 2010. Thus, mHDM/SCT leads to prolonged survival and favorable outcomes, especially if a hematologic CR is achieved.

Monoclonal gammopathy of undetermined significance in systemic transthyretin amyloidosis (ATTR)

Abstract Objective: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. Patients and methods: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016. Results: There were a total of 226 patients with ATTRwt and ATTR V122I amyloidosis evaluated during the specified time frame with 155 and 71 patients in each cohort, respectively. Those with complete medical records, 140 patients with ATTRwt and 57 V1221 ATTRm subjects, were included in the analyses. Fifty-five patients (39%) in the ATTRwt cohort and 28 patients (49%) in the ATTR V122I cohort had an MGUS, as indicated by an abnormality in the serum-free light-chain ratio and/or serum immunofixation electrophoresis. Conclusion: These data confirm the high prevalence of coexistent MGUS with ATTR amyloidosis in this patient population, with an MGUS rate that is higher than the general population. These findings also highlight the importance of a thorough diagnostic evaluation in patients with amyloidosis to determine the precursor protein, as the clinical course and treatment of AL (light-chain amyloid protein) and ATTR amyloidosis are distinct.

Review of siltuximab in the treatment of multicentric Castleman’s disease

Castleman’s disease (CD) is a rare lymphoproliferative disorder that has multiple histologic patterns, as well as two distinct clinical forms: unicentric or multicentric. Multicentric Castleman’s disease (MCD) may have mild symptoms in some cases, but in others it can progress to severe pancytopenia, life-threatening infection, secondary malignancy, multiorgan failure, or death. Recent research has determined that the etiology of the disease signs and symptoms is related to elevated cytokines, including interleukin 6 (IL-6). Siltuximab is a monoclonal antibody that targets IL-6 and is currently the only US Food and Drug Administration approved therapy for idiopathic MCD. Clinical data have demonstrated significant efficacy and tolerance of siltuximab in patients with idiopathic CD.

Vertebral compression fractures as the initial presentation of AL amyloidosis: case series and review of literature

Abstract The clinical presentation of AL amyloidosis is highly variable. In this series, we describe five cases of AL amyloidosis with vertebral compression fractures as initial presentation. All five patients had evidence of bone marrow replacement on magnetic resonance imaging and bone marrow biopsies demonstrating diffuse interstitial amyloid deposition. Hepatomegaly and elevated liver enzymes, consistent with liver involvement with amyloidosis, were also seen in each case. All five patients responded well to anti-plasma cell chemotherapy, with normalization of serum free light chain levels, reduction in alkaline phosphatase and improvement in pain and functional status. Although rare, AL amyloidosis should be considered in the differential diagnosis of selected patients with spontaneous vertebral compression fractures. Moreover, there seems to be an association of vertebral compression fractures with liver involvement in AL amyloidosis.

Tracking Breeds Success: Improved Retrieval Rates of Inferior Vena Cava Filters with Minimal Dedication of Resources.

measurement of stenosis was compared with the adjacent nonstenotic iliofemoral veins. If more than 50% cross sectional area or diameter reduction was found via IVUS imaging, it was treated with appropriate balloon size (range, 10 40-16 60) and stent (12-24 mm diameter by 40-90 mm length). Results: A total of 233 lesions were identified, with 115 in left lower extremity (LLE) and 118 in right lower extremity (RLE). The CEAP classification score in the LLE were C1, 0; C2, 35; C3, 40; C4, 15; C5, 20; C6, 6; with the most common site being proximal common iliac vein, 37.4% (20.86% females and 16.5% males). The CEAP classification score in the RLE were C1, 0; C2, 31; C3, 42; C4, 14; C5, 23; C6, 7; while most common site was middle external iliac vein, 31.35% (20.4% females and 11.01% males). The least common site of the NIVL was noted in LLE in the distal external iliac vein, 2.6% (2.6% females and 0% males). In the RLE, the least common site of NIVL was also in the distal external iliac vein, 7.62% (5.93% females and 1.69% males). No correlation between age, laterality, gender, or CEAP score has been noted. Conclusions: This analysis gives an insight into understanding the anatomical locations of the NIVL that are an often undiagnosed cause of lower extremity venous diseases. Despite multiple questions not yet answered, it gives an insight to clinicians and researchers to guide their treatment and research.