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Hematology Reports
Volume 8
Issue 3
10.4081/hr.2016.6625
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Case Report

Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab

by
Alexander G. Raufi
1,
Shruti Scott
1,
Omar Darwish
1,
Kevin Harley
1,
Kanwarpal Kahlon
1,
Sheetal Desai
1,
Yuxin Lu
2 and
Minh-Ha Tran
2,*
1
Department of Internal Medicine, University of California, Irvine, CA, USA
2
Department of Pathology and Laboratory Medicine, University of California, Irvine, UCIMC, Bldg. 1, Rm. 3003, Mail Code 4805, Irvine, CA 92697, USA
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2016, 8(3), 6625; https://doi.org/10.4081/hr.2016.6625
Submission received: 27 May 2016 / Revised: 11 September 2016 / Accepted: 23 September 2016 / Published: 30 September 2016

Abstract

Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs)–a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpuraa TMA classically associated with autoimmune depletion of ADAMTS-13–aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.
Keywords: atypical hemolytic uremic syndrome; microangiopathic hemolytic anemia; thrombotic microangiopathy; lupus nephritis atypical hemolytic uremic syndrome; microangiopathic hemolytic anemia; thrombotic microangiopathy; lupus nephritis

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MDPI and ACS Style

Raufi, A.G.; Scott, S.; Darwish, O.; Harley, K.; Kahlon, K.; Desai, S.; Lu, Y.; Tran, M.-H. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab. Hematol. Rep. 2016, 8, 6625. https://doi.org/10.4081/hr.2016.6625

AMA Style

Raufi AG, Scott S, Darwish O, Harley K, Kahlon K, Desai S, Lu Y, Tran M-H. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab. Hematology Reports. 2016; 8(3):6625. https://doi.org/10.4081/hr.2016.6625

Chicago/Turabian Style

Raufi, Alexander G., Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, and Minh-Ha Tran. 2016. "Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab" Hematology Reports 8, no. 3: 6625. https://doi.org/10.4081/hr.2016.6625

APA Style

Raufi, A. G., Scott, S., Darwish, O., Harley, K., Kahlon, K., Desai, S., Lu, Y., & Tran, M. -H. (2016). Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab. Hematology Reports, 8(3), 6625. https://doi.org/10.4081/hr.2016.6625

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