SUPERIOR VENA CAVA STENOSIS WITH PROXIMAL ESOPHAGEAL VARICES

NKF 2014 Spring Clinical Meetings Abstracts

53 SUPERIOR VENA CAVA STENOSIS WITH PROXIMAL ESOPHAGEAL VARICES: A RARE HEMODIALYSIS CATHETER RELATED COMPLICATION Dron Bhandari and Mala Sachdeva Hofstra North Shore-LIJ School of Medicine, Great Neck, NY Tunneled dialysis catheters have been associated with central vein stenosis, however rarely have been reported as causing superior vena cava (SVC) syndrome. Due to shunting of blood through the esophageal venous plexus, downhill or proximal esophageal varices can be formed. We report two cases of SVC stenosis presenting with SVC syndrome and life threatening gastrointestinal bleeding. Sixty -one year old male on hemodialysis presented with melena. Physical exam revealed swollen face, distended neck veins, and hypotension. Hemoglobin was 6.8mg/dl. Profuse hematemesis prompted emergent endoscopy which revealed proximal esophageal varices. Variceal formation was attributed to stenosis of the right brachiocephalic vein and right proximal SVC. He underwent temporary banding, angioplasty, and stenting of the SVC leading to resolution of the varices with no further bleeding episodes. Thirty- two year old female on hemodialysis presented with hematemesis. Exam was consistent with puffy face and distended neck veins. Emergent endoscopy revealed proximal esophageal varices. CT angiogram (CTA) revealed stenosis of the left brachiocephalic vein at the level of the SVC. She underwent temporary banding procedure and subsequent bypass graft from left axillary vein to right atrium which resulted in reduction of the esophageal varices and no further bleeding. SVC obstruction with proximal esophageal varices is a rare hemodialysis catheter -related complication but can become life threatening if gone untreated. Prompt recognition includes physical examination, endoscopy, and radiographic imaging such as CTA, MRI, or venography. Treatment options must be implemented in a timely fashion to reduce long term morbidity.

54 EXTRAMEDULLARY PLASMACYTOMA POST TRANSPLANT LYMPHOPROLIFERATIVE DISORDER (PTLD) IN A RENAL ALLOGRAFT TREATED WITH BORTEZOMIB Arpit Bhargava, Bhavna Chopra, Pritam Tayshetye, Katherine Jasnosz, Dulabh Monga, Richard Marcus. Allegheny General Hospital ,Pittsburgh, PA We present a case of a 59 year old woman who had a pediatric en bloc kidney transplant in 1988 on mycophenolate mofetil (MMF) and Cyclosporine with stable allograft function and a baseline creatinine (Cr) of 2.0 mg/ dl. MMF was discontinued in January 2013 because of recurrent skin cancer but cyclosporine continued. Serum and urine protein electrophoresis with immunofixation in February 2013 revealed an IgG kappa monoclonal gammopathy. Bone marrow (BM) biopsy did not reveal a plasma cell dyscrasia and a skeletal survey was negative for lytic lesions. Her creatinine gradually increased to 3.0 mg/dL. She had a kidney biopsy in September which revealed an atypical interstitial plasmacytic infiltrate (Fig1) with kappa light chain restriction. The biopsy also revealed Banff type 1A acute cellular rejection (Fig2). A repeat BM biopsy revealed 4% polyclonal plasma cells with no evidence of light chain restriction. The final diagnosis was extramedullary plasmacytoma PTLD. Her pretransplant EBV status is unknown but her EBV DNA in the renal biopsy was negative. The patient received Solumedrol for acute rejection and then was started on bortezomib and dexamethasone. She had a good initial response, with improved Cr to 2.0mg/dL and a decrease in free kappa light chains. Extramedullary plasmacytoma PTLD in allografts are extremely rare. Literature review revealed only 2 similar cases in the renal allograft where the patients required transplant nephrectomy. Our patient showed a good initial response to chemotherapy. In conclusion we report an extremely rare extramedullary renal allograft plasmacytoma which can be successfully treated.

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56 RENAL VASCULITIS WITH PULMONARY INVOLVEMENT IN AN IMMUNOSUPPRESSED PATIENT ON CHRONIC TREATMENT WITH HYDRALAZINE. Jimena Blandon, Beth Fromkin, Rute Paixao, Dianne Sandy, Mauro Braun, Cleveland Clinic Florida. Weston, Florida, USA Hydralazine is a commonly used anti-hypertensive that can cause drug-induced lupus erythematosus (DILE). Rarely it is reported to cause anti neutrophil cytoplasmic antibody (ANCA) positive-drug induced vasculitis (DIV). In the majority of cases it involves kidneys, skin, and lungs, infrequently associated with rapidly progressive glomerulonephritis (RPGN), and pulmonary renal syndrome is extremely rare. A 73 year old Indian male with CKD 3 and HTN on hydralazine for 4 years presented with 15 lbs weight gain, shortness of breath, back pain and generalized malaise. Initial BUN/creatinine 68/5.0 (baseline 34/2.0), peaked at 94/6.9 associated with nephrotic range proteinuria 6.27 gr. Cultures, hepatitis panel and HIV testing were negative. Negative paraproteinemia. ANA, anti-DS DNA, anti-histone antibody were positive with low complements. Renal biopsy reported immune complex mediated mesangio-proliferative GN. He required hemodialysis (HD), during which he developed hemoptysis and respiratory distress, leading to intubation. Plasmapheresis, high steroids pulse and IV cyclophosphamide were started. The patient was discharged depending of HD. DIV can present with multiple organ involvement. High doses of hydralazine increased the risk of this presentation. As hydralazine is widely used for treatment of HTN, DIV should be considered and prompt cessation may be sufficient to reverse it. Immunosupressants, HD and plasmapheresis may be needed for definitive treatment.

Am J Kidney Dis. 2014;63(5):A1-A121