Typical Pregnancy Before Atypical Syndrome?

NKF 2016 Spring Clinical Meetings Abstracts

Case Report 373 TRICLONAL GAMMOPATHY, HIV AND CKD: A Verma, M Gupta, R Thabet, J Oshea, Helmut G Rennke MD. St Vincent Hospital, Worcester, MA, USA. Triclonal Gammopathy (TG) is an immunoglobulin (Ig) abnormality where three discrete monoclonal subpopulations of Ig molecules are present in a patient. Unlike biclonal gammopathy seen in 4 percent of patients with monoclonal paraproteinemia (MP), TG is an exceedingly rare entity. We report an unprecedented case of a 41-year-old Ghanaian female with hypertension and worsening kidney function found to have TG. Upon initial evaluation for chronic kidney disease (CKD), autoimmune & hepatitis screen were negative, imaging was normal but she had an IgG/Lambda MP on serum immunofixation (IF). Bone marrow biopsy ruled out myeloma. CKD was thus attributed to hypertensive nephrosclerosis. Over the next year, her creatinine doubled. A kidney biopsy showed mild mesangioproliferative injury and severe arterial & arteriolar sclerosis. Immunoflourescence showed hitherto unreported deposition of two MPs in the glomeruli - IgG/Kappa 1 and IgG/Kappa 2. Thus this patient with another MP in the serum had TG. A mammogram, bone scan and CT chest, abdomen & pelvis were negative. HIV was positive. She will be kept in close follow up with periodic screening for malignancy as it may manifest years after detection of the TG. Patient’s kidney failure is worsening and she will soon initiate dialysis. This case is unique due to TG’s manifestation in a young patient and the detection of two MPs in the kidney. The three MP clones were from the same Ig class – IgG. They likely arose from the same B-cell clone that maintained their variable region specificity but switched heavy chain classes leading to generation of 3 distinct paraproteins. An occult lymphoproliferative disorder or a plasma cell dyscrasia has led to generation of TG in this patient. HIV infection maybe contributing too but unlikely as neither the pattern of kidney injury nor the unique restriction of Ig deposition is consistent with HIV nephropathy or postinfectious glomerulonephritis. Almost all the reported cases of TG have a lymphoproliferative disorder; a few had non-hematological conditions and three had TG of unknown significance. Our report adds to the limited literature on TG and the evolving data from this and other contemporary patients, will hopefully laydown guidelines for optimum management of future patients.

374 MASS SAFETY CHECKS IN HEMODIALYSIS PATIENTS DURING NATURAL AND HUMANITARIAN DISASTERS: Divya Vijayan1, Kavita Vijayan1, Stephanie Batarseh1, Vijay Lapsia2.1Bergen County Academies, New Jersey. 2Ichan School of Medicine at Mount Sinai, New York, USA. During disasters, patients on hemodialysis receiving in-center hemodialysis (HD) are vulnerable and disruptions can result in missed treatments. We identified available tools that can potentially facilitate mass safety checks on hemodialysis patients. Using a keyword web search we identified all documented applications of informatics tools used during massive disasters. We found several tools used for effective mass communication in multiple instances of natural and humanitarian disasters: 1. FEMA App: During hurricane Sandy, FEMA received more than 10,000 requests in one day from people searching for shelter locations within a specific ZIP code. 2. Person Finder by Google: Directory and message board used to locate people during various disasters including the Haiti earthquake and Boston bombings. 3. Safety Check by Facebook: Activated during several major natural disasters. Most recently widely used during the Paris and Nigerian terrorist attacks. 4. Twitter Short Messages & SOS service: Twitter messages have been used extensively during multiple natural and humanitarian disasters. Twitter SOS service developed with the Indian army was successfully used to identify and save over 12,000 people during Kashmir floods. 5. Safe and Well by American Red Cross: Initially used during the Washington landslides to help families connect, the service can be used to post safe and well messages. 6. Signal Messaging application: National Grid deployed Signal for mass communication during Hurricane Irene. These web based tools were successful in relaying information, helping locate survivors, and funnel critically needed resources. Web based technology has become a powerful tool during disaster response, when traditional technologies are generally unable to cope with the demand in the immediate aftermath of a crisis. Safety checks in chronic hemodialysis patients using social media and mobile apps may be a viable alternative to identify and locate patients that need the most help. These tools may facilitate targeted intervention reducing morbidity and mortality in this susceptible population.

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AGGRESSIVE B-CELL NHL PRESENTING AS ACUTE KIDNEY INJURY Sharad Virmani. Georgia Nephrology, LLC, Lawrenceville, Georgia, USA Diffuse Large B-Cell Lymphoma (DLBCL) is the prominent form of NonHodkin Lymphoma. While renal involvement can be common in advanced stages, presentation as acute kidney injury is an atypical presentation. Laith Al-Rabadi, MBBS,1,* Rivka A 71 y/o white female with hypertension presented to her primary care physician with general weakness and fatigue for 1 month. Two daysE. laterBallard, she Jennifer MD,2,y Alan was asked to report to the Emergency Room for “abnormal kidney labs.” Her David J. Salant, MD,1 Bun/CR was 89 and 4.67mg/dl, GFR of 9ml/min, and a WBC of 8.9 with Hct of 25.8%. Her labs from one year prior were “normal.” Her initial serology for autoimmune etiology for AKI were within normal limits. FeNa was > 1% and is little information about pregnancy o her urinalysis revealed sterile pyuria and thus, sheThere was initiated on IVF with isotonic fluids and started on IV solumederol with a transition those to oral with circulating autoantibod especially prednisone for a presumptive diagnosis of interstitial nephritis due to remote MN. We present what autoantigen in primary and acute history of NSAID use. The renal ultrasound indicated right kidney ofwith PLA R-associate a 39-year-old woman 2 12.4 cm and left kidney of 13.3cm (Her height was 55 inches). Her GFR anasarca, hypoalbuminemia (albumin, 1.3-2. improved only to 19ml/min and a kidney biopsy was pursued. The five opsy revealed glomeruli appeared normal, severe interstitial infiltration of atypical MN with staining for PLA2R, a She did not respond to conservative therapy a lymphocytes with monocytes with mild inflammation. Immunohistochemistry indicated positive stains for CD45, CD20, BCL-6, BCL-2, and MYC after (70%).presentation, she was fou Several weeks Unfortunately no IF as glomeruli sample was limited. Hematology was further immunosuppressive treatment. Protei consulted for a diagnosis of Diffuse B-Cell Lymphoma. PET-CT scan indicated Circulating anti-PLA2R levels declined but w involvement in the pancreas, right adrenal, osseous structures of the Thoracic without spine and iliac, tissue fullness in the both kidneys. Bone proteinuria biopsy revealedat birth or at her subseque had detectable anti-PLA2R of imm normocellular marrow without lymphomatous involvement. Stage IVcirculating B-Cell NHL was established and R-CHOP(Rituximab, low Cytoxan,Vincristine, titers. Only trace amounts of IgG4 ant Doxorubicin, Prednisone. She is currently on her 3 cycle of chemotherapy discrepancy betweenand anti-PLA2R levels in th is feeling well. Am J Kidney Dis. 67(5):775-778. ª 2016 by The presentation of AKI with enlarged kidneys on ultrasound can be tell tale sign of lymphoma or an infiltrative process like Amyloidosis. While primary INDEX nephropathy ( renal lymphoma is rare, aggressive and advanced forms WORDS: of B-Cell NHLMembranous can receptor (PLA involve the kidney parenchyma and present with low GFR and index 2R); autoantibody; placenta; ritu complications of AKI. The effective form of treatment is chemotherapy targeted toward the lymphoma. This case highlights the importance of kidney biopsy with the appropriate Immunohistochemistry stainings with suspicious atypical lymphocytes in the interstium. regnant patients with autoimmune disease

Pregnancy in a Patient Wit and Circulating Anti-P

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deliver newborns with a spectrum of cl manifestations due to the transplacental passa 376 circulating autoantibodies. Pregnant patients TYPICAL PREGNANCY BEFORE ATYPICAL SYNDROME? Ritu Ramdeo Vyas, Ami Mlupus Patel or myasthenia gravis can deliver babies 1,2 corresponding disease Neo University of Maryland Medical Center, Baltimore, MD, USAin the neonate. Hemolytic Uremic Syndrome (HUS) is commonly associated(MN) with not associated membranous nephropathy diarrhea due to Shiga-toxin. Recently, more cases ofwas atypical HUS congenital infection first described in 199 (aHUS); 6-7% of all HUS; have been recognized to be caused by comattributed to the passive transfer of maternal plement dysregulation. Triggers like pregnancy and infection can acti- 3 bodies to putative renal antigens. More than a d vate the alternative pathway, which damages renal endothelium, actilater, et al4 identified the first antigen inv vating coagulation cascade and Debiec causing microthrombi and hence, thrombotic microangiopathy on the underin(TMA). suchTreatment cases depends as neutral endopeptidase (NE lying physiology. Herein, we present a case of TMA. metalloprotease present on the surface of the pod 26-year-old female, 18 weeks pregnant, with an uncomplicated pregand involved in the proteolytic regulation of va nancy two years ago and no known history of chronic kidney disease, peptides. Debiec et alShe described a mother w presented to the emergencytive department with vaginal bleeding. had mutation who had fo intra-uterine fetal demise and was noted preventing to have anemia NEP (Hb 6.7expression g/dL) , thrombocytopenia (plateletsanti-NEP 34,000/µl) and acute kidneydue injuryto(AKI; antibodies fetomaternal alloi creatinine 2 mg/dL). Initial thought was disseminated intravascular nization from a previous miscarriage; these antib coagulopathy. She underwent dilatation and curettage (D&C). Subsewere to cross the placenta and cause subepit quently, her creatinine increased to 5.8 mg/dl over a four day period. deposits into the fetal of a subsequent Anemia and thrombocytopenia also failed improve post kidney D&C. Blood nancy. M-type smear showed multiple schistocytes and dailyphospholipase plasmapheresis wasA2 receptor (PL started. Anemia, thrombocytopenia and identified AKI improved fivemajor days. autoantigen fo was later as inthe ADAMTS 13 and complement levels (C3, C4 and CH50)5were normal. mary MN in adults. Little literature exists Complement factor H and I were elevated. pregnancy outcomes in patients with nephrotic In our case, patient was thought to have aHUS, which is rare in padrome duehistory to primary MN, with no data ava tients with no known personal or family and previous normal pregnancy. While most of the reported cases with complement about pregnancy in PLA2mediatR-associated disease ed aHUS need terminal complement Eculizumab, present inhibitor, what we believeour to patient be the first known ca responded to plasmapheresis only. pregnancy in a patient with PLA2R-associated who was seropositive for anti-PLA2R autoantib throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118