@article{d581e11fafd445149fd98573daa67f13,
title = "A rare case of renal thrombotic microangiopathy associated with Castleman's disease",
abstract = "Background: Castleman's disease (CD) is an uncommon, heterogeneous lympho-proliferative disorder leading to high circulating levels of interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). Renal involvement has been only described in a limited number of small studies. Herein, we report a rare case of renal thrombotic microangiopathy (TMA) associated with CD and investigate the podocyte expression of VEGF in the renal biopsy prior to initiation of treatment. Case presentation: An 18-year-old male presented with fever, diarrhea, diffuse lymphadenopathy, ascites and acute kidney injury. Laboratory tests for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura were negative. The kidney biopsy showed TMA. An excisional lymph node biopsy was consistent with CD, plasma cell variant. Immunofluorescence staining showed suppressed podocyte VEGF expression. Chemotherapy that inhibits production of inflammatory mediators including IL-6 and VEGF led to complete recovery of renal function. Conclusions: Our case illustrates a rare renal histological feature of CD. IL-6 and VEGF are postulated to suppress glomerular VEGF expression, thereby causing renal TMA. Therapy directed against these inflammatory mediators may have important therapeutic implications.",
keywords = "Castleman's disease, Podocytes, Thrombotic microangiopathy, VEGF",
author = "Anubha Mutneja and Cossey, {L. Nicholas} and Helen Liapis and Chen, {Ying Maggie}",
note = "Funding Information: Y.M.C. is supported by the National Institutes of Health K08DK089015 and R03DK106451, Halpin Foundation-American Society of Nephrology Research Grant, Faculty Scholar Award (MD-FR-2013-336) from the Children{\textquoteright}s Discovery Institute of Washington University and St. Louis Children{\textquoteright}s Hospital, Clinical Scientist Development Award (Grant 2015100) from the Doris Duke Charitable Foundation, Career Development Award from Nephrotic Syndrome Study Network (NEPTUNE), Early Career Development Award from Central Society for Clinical and Translational Research (CSCTR) and Renal Translational Innovation Grant from Washington University Division of Nephrology. Y.M.C. is a member of Washington University Diabetes Research Center (supported by NIH P60 DK020579), Washington University Musculoskeletal Research Center (supported by NIH P30AR057235), and Washington University Institute of Clinical and Translational Sciences (UL1 TR000448). Part of this material was presented in a Poster at the 2014 Annual Meeting of the American Society of Nephrology; November 13 through 16, 2014; Philadelphia, PA. Publisher Copyright: {\textcopyright} 2017 The Author(s).",
year = "2017",
month = feb,
day = "10",
doi = "10.1186/s12882-017-0472-2",
language = "English",
volume = "18",
journal = "BMC Nephrology",
issn = "1471-2369",
number = "1",
}