{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T11:24:47Z","timestamp":1761218687623,"version":"3.41.2"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T00:00:00Z","timestamp":1723507200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Nephrol."],"abstract":"<jats:p>Thrombotic microangiopathy (TMA) is a rare renal complication of patients with chronic lymphocytic leukemia (CLL) and is often associated with peripheral features. We present the first case of CLL patients with renal-limited TMA. A 70-year-old female patient with a history of well-controlled type 2 diabetes and baseline albuminuria of 87.2 mg\/g 1 year prior and CLL was on active surveillance only. Her baseline white blood cell (WBC) was 202.6 x 10<jats:sup>3<\/jats:sup>\/\u00b5l. She presented with nephrotic syndrome with proteinuria of 10 g\/g and a subsequent unremarkable serologic work-up. A kidney biopsy revealed diabetic glomerulosclerosis and chronic TMA. Initially, she was treated conservatively with angiotensin receptor blockade and sodium glucose cotransporter-2 inhibition but progressed with increased proteinuria of 17 g\/g. Complement functional panel testing was pursued and showed dysregulation of the classical and alternative complement pathways. We decided to treat CLL which was suspected to be the culprit. At 9 months post-ibrutinib initiation, there was a 90% reduction in the WBC as well as a 94% reduction in proteinuria (17 g\/g to 0.97 g\/g). This case emphasizes the role of complement dysregulation in the pathogenesis of TMA in CLL patients. Treatment of CLL can restore complement dysregulation and improve renal outcomes.<\/jats:p>","DOI":"10.3389\/fneph.2024.1400027","type":"journal-article","created":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T05:10:21Z","timestamp":1723525821000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case report: Successful treatment of renal-limited thrombotic microangiopathy secondary to chronic lymphocytic leukemia"],"prefix":"10.3389","volume":"4","author":[{"given":"Kristina","family":"Nasr","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabine","family":"Karam","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marshall","family":"Mazepa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jan","family":"Czyzyk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nattawat","family":"Klomjit","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,8,13]]},"reference":[{"volume-title":"Chronic lymphocytic leukemia","year":"2023","author":"Mukkamalla","key":"B1"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2015.128793","article-title":"Renal complications in chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis: the Mayo Clinic experience","volume":"100","author":"Strati","year":"2015","journal-title":"Haematologica"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.13954","article-title":"Diagnosis and treatment of thrombotic microangiopathy","author":"Thompson","year":"2022","journal-title":"Int J Lab Hematol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1053\/j.ajkd.2022.10.014","article-title":"Renal thrombotic microangiopathy: A review","volume":"81","author":"Genest","year":"2023","journal-title":"Am J Kidney Dis May"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2022.813439","article-title":"Case report: chronic lymphocytic leukemia with recurrent complement-mediated thrombotic microangiopathy and C3 glomerulonephritis","volume":"9","author":"Ma","year":"2022","journal-title":"Front Med (Lausanne)"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1155\/2023\/7803704","article-title":"A rare case of renal thrombotic microangiopathy and focal segmental glomerulosclerosis secondary to plasma cell leukemia","volume":"2023","author":"Komisarof","year":"2023","journal-title":"Case Rep Hematol"},{"key":"B7","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1136\/jcp.2005.035592","article-title":"Distinguishing diabetic nephropathy from other causes of glomerulosclerosis: an update","volume":"60","author":"Alsaad","year":"2007","journal-title":"J Clin Pathol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26705","article-title":"The prevalence and clinical outcomes of microangiopathic hemolytic anemia in patients with biopsy-proven renal thrombotic microangiopathy","volume":"97","author":"Bhutani","year":"2022","journal-title":"Am J Hematology"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1093\/ckj\/sfy026","article-title":"Renal involvement in chronic lymphocytic leukemia","volume":"11","author":"Wanchoo","year":"2018","journal-title":"Clin Kidney J"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1177\/23993693221079644","article-title":"Chronic lymphocytic leukemia and light chain proximal tubulopathy: A rare presentation of a common disease","volume":"6","author":"Klomjit","year":"2022","journal-title":"J Onco-Nephrology"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.11470918","article-title":"Etiology and outcomes of thrombotic microangiopathies","volume":"14","author":"Bayer","year":"2019","journal-title":"Clin J Am Soc Nephrol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.27101","article-title":"Frequency and characteristics of chemotherapy-associated thrombotic microangiopathy: Analysis from a large pharmacovigilance database","volume":"98","author":"Klomjit","year":"2023","journal-title":"Am J Hematol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2016.09.045","article-title":"Thrombotic microangiopathy associated with monoclonal gammopathy","volume":"91","author":"Ravindran","year":"2017","journal-title":"Kidney Int"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.780107","article-title":"Thrombotic microangiopathy, an unusual form of monoclonal gammopathy of renal significance: report of 3 cases and literature review","volume":"12","author":"Filippone","year":"2021","journal-title":"Front Immunol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1002\/ajh.2830190109","article-title":"Defective complement activity in chronic lymphocytic leukemia","volume":"19","author":"Heath","year":"1985","journal-title":"Am J Hematol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.603569","article-title":"The role of alpha 2 macroglobulin in igG-aggregation and chronic activation of the complement system in patients with chronic lymphocytic leukemia","volume":"11","author":"Naseraldeen","year":"2020","journal-title":"Front Immunol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1038\/s41581-018-0077-4","article-title":"The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group","volume":"15","author":"Leung","year":"2019","journal-title":"Nat Rev Nephrol Jan"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.tranon.2021.101081","article-title":"Thrombotic microangiopathy in oncology - a review","volume":"14","author":"Val\u00e9rio","year":"2021","journal-title":"Transl Oncol"},{"key":"B19","first-page":"483","article-title":"Ibrutinib (imbruvica): a novel targeted therapy for chronic lymphocytic leukemia","volume":"39","author":"Parmar","year":"2014","journal-title":"P t"}],"container-title":["Frontiers in Nephrology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneph.2024.1400027\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T05:10:24Z","timestamp":1723525824000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneph.2024.1400027\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,13]]},"references-count":19,"alternative-id":["10.3389\/fneph.2024.1400027"],"URL":"https:\/\/doi.org\/10.3389\/fneph.2024.1400027","relation":{},"ISSN":["2813-0626"],"issn-type":[{"type":"electronic","value":"2813-0626"}],"subject":[],"published":{"date-parts":[[2024,8,13]]},"article-number":"1400027"}}