ReviewInternational urology and nephrology2016
Hemophagocytic lymphohistiocytosis: an update for nephrologists.
Review in International urology and nephrology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 26 citations in OpenAlex.
- Clinical spectrum and laboratory correlates of concurrent hemophagocytic lymphohistiocytosis and toxoplasmosis: a systematic review.BMC infectious diseases · 2026Pooled it
- Discrepant Urinary Findings Between Dipstick Protein and Sulfosalicylic Acid Test in a Patient With Hyaline Casts: A Case Report.Journal of clinical laboratory analysis · 2026Article
- Body mass index and hemophagocytic lymphohistiocytosis: a risk assessment in HIV-positive leukemia patients.Annals of medicine and surgery (2012) · 2025Review
- Hemophagocytic Lymphohistiocytosis in Solid Organ Transplants.Kidney international reports · 2025Article
- Nephrotoxicity in Bispecific Antibodies Recipients: Focus on T-Cell-Engaging Bispecific Antibodies.OncoTargets and therapy · 2024Review
- Early response markers predict survival after etoposide-based therapy of hemophagocytic lymphohistiocytosis.Blood advances · 2023Article
- Review
- Renal involvement as a unique manifestation of hemophagocytic syndrome.Frontiers in medicine · 2022Article
- Bile Cast Nephropathy Secondary to Hemophagocytic Lymphohistiocytosis With Liver Failure.Cureus · 2020Article
- Secondary hemophagocytic syndrome after renal transplantation: two case-reports.Jornal brasileiro de nefrologia · 2020Article
- A young child with fever and unexplained acute kidney injury: Answers.Pediatric nephrology (Berlin, Germany) · 2018Article
- Minimal Change Disease as Initial Presentation of ALK-Positive Anaplastic Large-Cell Lymphoma in a Pediatric Patient.Case reports in oncologyArticle
- Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by defective lytic capability of cytotoxic T lymphocytes and NK cells, which results in proliferation of benign hemophagocytic histiocytes. A cytokine storm ensues, and a severe systemic inflammatory response syndrome, multiorgan dysfunction syndrome, and death frequently follow. It may occur as a primary (inherited) form, or be acquired secondary to malignancy, infection, rheumatologic disease, or immunosuppression. Cardinal manifestations include fever, cytopenias, hepatosplenomegaly, and dysfunction of liver, kidney, CNS, and/or lung. Additional laboratory findings include marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, abnormal LFTs, coagulopathy, and hyponatremia. Nephrologists need to be aware of this syndrome owing to the frequent occurrence of acute kidney injury in these severely ill patients. Glomerulopathy and nephrotic syndrome may develop. Kidney transplant recipients are at increased risk of HLH due to immunosuppression, and most such cases are triggered by infection with over 50 % mortality. Effective treatment of HLH usually requires chemoimmunotherapy to acutely suppress inflammation, specific treatment of underlying infection or malignancy, and in certain cases hematopoietic stem cell transplantation. The pathogenesis, clinical manifestations, diagnosis, and treatment of HLH are discussed.
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