Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-02-01
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| Series: | Kidney International Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468024923015759 |
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| author | Miroslav Sekulic Ibrahim Batal Satoru Kudose Dominick Santoriello M. Barry Stokes Belinda Jim Hans-Peter Marti Øystein Eikrem Jai Radhakrishnan Vivette D. D’Agati Glen S. Markowitz |
| author_facet | Miroslav Sekulic Ibrahim Batal Satoru Kudose Dominick Santoriello M. Barry Stokes Belinda Jim Hans-Peter Marti Øystein Eikrem Jai Radhakrishnan Vivette D. D’Agati Glen S. Markowitz |
| author_sort | Miroslav Sekulic |
| collection | DOAJ |
| description | Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes in the kidney are limited. Methods: To characterize kidney pathology in HLH, a retrospective review of 30 patients’ clinical and laboratory data, and kidney tissue was performed (18 from autopsy, and 12 biopsied patients). Results: HLH was associated with infection (83%), autoimmune disease (37%), and malignancy (20%), including 30% with concurrent autoimmune disease and infection. Nephrological presentations included subnephrotic range proteinuria (63%), AKI (63%), hematuria (33%), chronic kidney disease (CKD, 20%), nephrotic range proteinuria (13%), and nephrotic syndrome (7%); and 40% of patients required hemodialysis (HD). Among the 12 patients who underwent kidney biopsy, 6 subsequently showed improved kidney function and the remainder had progressive CKD with most progressing to end-stage kidney disease. Autopsy patients had a median terminal admission of 1 month, and 33% of the biopsied patients died (ranging from 0.3–5 months post-biopsy). Variable pathologies were identified, including acute tubular injury (ATI, 43%), lupus nephritis (LN, 23%), collapsing glomerulopathy (17%), thrombotic microangiopathy (TMA, 17%), and cortical necrosis (10%). Most autopsied patients had significant kidney pathology other than ATI that likely contributed to kidney function decline. A majority of patients with HLH exhibited kidney dysfunction that likely contributed to the poor prognosis. Conclusion: Kidney dysfunction in HLH should not be assumed to be solely attributable to ATI, and in certain scenarios a kidney biopsy may be warranted. |
| format | Article |
| id | doaj-art-6f8d99fddaca4735bd435abbbda4a275 |
| institution | Kabale University |
| issn | 2468-0249 |
| language | English |
| publishDate | 2024-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Kidney International Reports |
| spelling | doaj-art-6f8d99fddaca4735bd435abbbda4a2752025-08-20T03:45:06ZengElsevierKidney International Reports2468-02492024-02-019241042210.1016/j.ekir.2023.10.033Kidney Dysfunction and Pathology in the Setting of Hemophagocytic LymphohistiocytosisMiroslav Sekulic0Ibrahim Batal1Satoru Kudose2Dominick Santoriello3M. Barry Stokes4Belinda Jim5Hans-Peter Marti6Øystein Eikrem7Jai Radhakrishnan8Vivette D. D’Agati9Glen S. Markowitz10Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA; Correspondence: Miroslav Sekulic, Department of Pathology and Cell Biology, Columbia University Irving Medical Center, 630 West 168th Street, VC14-238C, New York, New York 10032, USA.Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USADepartment of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USADepartment of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USADepartment of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USADepartment of Medicine, Division of Nephrology, Jacobi Medical Center at Albert Einstein College of Medicine, New York, New York, USADepartment of Clinical Medicine, University of Bergen, Bergen, Norway; Department of Medicine, Haukeland University Hospital, Bergen, NorwayDepartment of Clinical Medicine, University of Bergen, Bergen, Norway; Department of Medicine, Haukeland University Hospital, Bergen, NorwayDepartment of Internal Medicine, Nephrology Division, Columbia University Irving Medical Center, New York, New York, USADepartment of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USADepartment of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USAIntroduction: Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome produced by dysregulated activation of the immune system. Acute kidney injury (AKI) and proteinuria have been infrequently described in the setting of HLH, and investigations of underlying histopathologic changes in the kidney are limited. Methods: To characterize kidney pathology in HLH, a retrospective review of 30 patients’ clinical and laboratory data, and kidney tissue was performed (18 from autopsy, and 12 biopsied patients). Results: HLH was associated with infection (83%), autoimmune disease (37%), and malignancy (20%), including 30% with concurrent autoimmune disease and infection. Nephrological presentations included subnephrotic range proteinuria (63%), AKI (63%), hematuria (33%), chronic kidney disease (CKD, 20%), nephrotic range proteinuria (13%), and nephrotic syndrome (7%); and 40% of patients required hemodialysis (HD). Among the 12 patients who underwent kidney biopsy, 6 subsequently showed improved kidney function and the remainder had progressive CKD with most progressing to end-stage kidney disease. Autopsy patients had a median terminal admission of 1 month, and 33% of the biopsied patients died (ranging from 0.3–5 months post-biopsy). Variable pathologies were identified, including acute tubular injury (ATI, 43%), lupus nephritis (LN, 23%), collapsing glomerulopathy (17%), thrombotic microangiopathy (TMA, 17%), and cortical necrosis (10%). Most autopsied patients had significant kidney pathology other than ATI that likely contributed to kidney function decline. A majority of patients with HLH exhibited kidney dysfunction that likely contributed to the poor prognosis. Conclusion: Kidney dysfunction in HLH should not be assumed to be solely attributable to ATI, and in certain scenarios a kidney biopsy may be warranted.http://www.sciencedirect.com/science/article/pii/S2468024923015759autoimmune diseasehemophagocytic lymphohistiocytosishistopathologyinfectionmalignancy |
| spellingShingle | Miroslav Sekulic Ibrahim Batal Satoru Kudose Dominick Santoriello M. Barry Stokes Belinda Jim Hans-Peter Marti Øystein Eikrem Jai Radhakrishnan Vivette D. D’Agati Glen S. Markowitz Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis Kidney International Reports autoimmune disease hemophagocytic lymphohistiocytosis histopathology infection malignancy |
| title | Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis |
| title_full | Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis |
| title_fullStr | Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis |
| title_full_unstemmed | Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis |
| title_short | Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis |
| title_sort | kidney dysfunction and pathology in the setting of hemophagocytic lymphohistiocytosis |
| topic | autoimmune disease hemophagocytic lymphohistiocytosis histopathology infection malignancy |
| url | http://www.sciencedirect.com/science/article/pii/S2468024923015759 |
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