ArticleJournal of clinical immunology2020
Thinking Beyond HLH: Clinical Features of Patients with Concurrent Presentation of Hemophagocytic Lymphohistiocytosis and Thrombotic Microangiopathy.
Article in Journal of clinical immunology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 35 papers.
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Who cites it
35 citing papers in PubMed, 52 citations in OpenAlex.
- Trial
- Multisystem inflammation and susceptibility to viral infections in human ZNFX1 deficiency.The Journal of allergy and clinical immunology · 2021Trial
- Hemophagocytic lymphohistiocytosis in critically ill patients: A guide for clinicians.World journal of critical care medicine · 2026Review
- Defibrotide for Protecting Against and Managing Endothelial Injury in Hematologic Malignancies and COVID-19.Biomolecules · 2025Review
- The complement system in autoimmune diseases: pathogenesis, diagnostic markers, and therapeutic strategies.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2025Review
- C3 as a predictive and prognostic biomarker in adult hemophagocytic lymphohistiocytosis: a large cohort study in China.Blood advances · 2025Article
- Case Report: Life-threatening overlap of hemophagocytic syndrome and atypical hemolytic uremic syndrome in a patient with autoimmune polyglandular syndrome type 1 successfully treated with targeted immunotherapy.Frontiers in immunology · 2025Article
- Identification of iron metabolism-related key genes and exploration of their potential mechanisms in hemophagocytic lymphohistiocytosis based on transcriptome sequencing.Frontiers in medicine · 2025Article
- Macrophage activation syndrome successfully treated with eculizumab and emapalumab: a case report.Frontiers in immunology · 2025Article
- Genetics of progressive multifocal leukoencephalopathy: update on case reports with an inborn error of immunity and risk variants found in drug-linked cases.Frontiers in neurology · 2025Review
- Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis.Kidney international reports · 2024Article
- Hemophagocytic lymphohistiocytosis is associated with deficiency and closed conformation of ADAMTS-13.Research and practice in thrombosis and haemostasis · 2024Article
- Early response markers predict survival after etoposide-based therapy of hemophagocytic lymphohistiocytosis.Blood advances · 2023Article
- Where have all the platelets gone? HIT, DIC, or something else?Hematology. American Society of Hematology. Education Program · 2023Review
- Population-level single-cell genomics reveals conserved gene programs in systemic juvenile idiopathic arthritis.The Journal of clinical investigation · 2023Article
- Proteomic Analysis of Pediatric Hemophagocytic Lymphohistiocytosis: a Comparative Study with Healthy Controls, Sepsis, Critical Ill, and Active Epstein-Barr virus Infection to Identify Altered Pathways and Candidate Biomarkers.Journal of clinical immunology · 2023Article
- Emapalumab for the treatment of refractory cytokine release syndrome in pediatric patients.Blood advances · 2023Article
- The Liver in Hemophagocytic Lymphohistiocytosis: Not an Innocent Bystander.Journal of pediatric gastroenterology and nutrition · 2023Review
- The successful use of eculizumab for treatment of thrombotic microangiopathy in pediatric acute SARSCoV2 infection and multisystem inflammatory syndrome in children.Haematologica · 2022Article
- The Role of Complement in HSCT-TMA: Basic Science to Clinical Practice.Advances in therapy · 2022Review
Corrections and comments
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Authors and funding
10 authors at 3 institutions in 1 country.
Funding
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune system activation driven mainly by high levels of interferon gamma. The clinical presentation of HLH can have considerable overlap with other inflammatory conditions. We present a cohort of patients with therapy refractory HLH referred to our center who were found to have a simultaneous presentation of complement-mediated thrombotic microangiopathy (TMA). Twenty-three patients had therapy refractory HLH (13 primary, 4 EVB-HLH, 6 HLH without known trigger). Sixteen (69.6%) met high-risk TMA criteria. Renal failure requiring renal replacement therapy, severe hypertension, serositis, and gastrointestinal bleeding were documented only in patients with HLH who had concomitant complement-mediated TMA. Patients with HLH and without TMA required ventilator support mainly due to CNS symptoms, while those with HLH and TMA had respiratory failure predominantly associated with pulmonary hypertension, a known presentation of pulmonary TMA. Ten patients received eculizumab for complement-mediated TMA management while being treated for HLH. All patients who received the complement blocker eculizumab in addition to the interferon gamma blocker emapalumab had complete resolution of their TMA and survived. Our observations suggest co-activation of both interferon and complement pathways as a potential culprit in the evolution of thrombotic microangiopathy in patients with inflammatory disorders like refractory HLH and may offer novel therapeutic approaches for these critically ill patients. TMA should be considered in children with HLH and multi-organ failure, as an early institution of a brief course of complement blocking therapy in addition to HLH-targeted therapy may improve clinical outcomes in these patients.
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Registered trials
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