ReviewTherapeutics and clinical risk management2021
Clinical Management of Relapsed/Refractory Hemophagocytic Lymphohistiocytosis in Adult Patients: A Review of Current Strategies and Emerging Therapies.
Review in Therapeutics and clinical risk management, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 34 citations in OpenAlex.
- Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis in the Gulf Cooperation Council: a modified Delphi approach.Frontiers in immunology · 2026Article
- Meta-analysis of tuberculosis incidence and risk in cancer patients treated with immune checkpoint inhibitors.Frontiers in oncology · 2026Article
- Presentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.Cureus · 2025Article
- Promising Targeted Therapies for Hemophagocytic Lymphohistiocytosis: A Translational Perspective Based on Immunopathology.Clinical reviews in allergy & immunology · 2025Review
- Diagnostic Challenges in a Case of Hemophagocytic Lymphohistiocytosis Likely Induced by Coxiella burnetii.Cureus · 2025Article
- Primary prostatic Burkitt's lymphoma complicated with hemophagocytic lymphohistiocytosis: a case report and literature review.Frontiers in oncology · 2025Article
- Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms.Journal of hematology & oncology · 2024Review
- Salvage treatment of ruxolitinib for refractory adenovirus-associated hemophagocytic syndrome post-haploidentical allogeneic stem cell transplantation: a case report.Translational pediatrics · 2024Article
- Real-world treatment patterns and outcomes in patients with primary hemophagocytic lymphohistiocytosis treated with emapalumab.Blood advances · 2024Article
- Hemophagocytic Lymphohistiocytosis Associated With Polyserositis and Salmonella typhi Infection.Cureus · 2023Article
- Case report of extranodal natural killer/T-cell lymphoma that induced secondary hemophagocytic syndrome-related histiocytic glomerulopathy.The Journal of international medical research · 2023Article
- Perioperative Management of Recurrent Hemophagocytic Syndrome in a Pregnant Woman: A Case Report.The American journal of case reports · 2023Article
- The diagnostic importance of multiple cytokines in adult hemophagocytic lymphohistiocytosis.Journal of clinical laboratory analysis · 2023Article
- Ruxolitinib as an Effective Treatment for Hemophagocytic Lymphohistiocytosis Secondary to SARS-Cov-2 Infection: A Case Report.Infection and drug resistance · 2023Article
- Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19.Frontiers in immunology · 2023Review
- Haemophagocytic lymphohistiocytosis (HLH) in an adult following treatment with trimethoprim/sulfamethoxazole.BMJ case reports · 2022Article
- Hemophagocytic Lymphohistiocytosis secondary to Rickettsial infection: A case report.Clinical case reports · 2022Article
- Systems biology approach reveals a common molecular basis for COVID-19 and non-alcoholic fatty liver disease (NAFLD).European journal of medical research · 2022Article
- Short-term, low-dose etoposide in refractory adult-onset Still's disease-associated macrophage activation syndrome.Clinical rheumatology · 2022Article
- The efficacy and safety of allogeneic stem cell transplantation in Mevalonate Kinase Deficiency.Pediatric rheumatology online journal · 2022Article
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionHaemophagocytic lymphohistiocytosis (HLH) is a severe disorder with high mortality. The aim of this review is to update clinical management of relapsed/refractory HLH in adults, with a focus on current and new therapies.
methodsWe searched relevant articles in Embase and PUBMED with the MESH term "hemophagocytic lymphohistiocytosis; refractory; relapsing; adult."
resultsOne hundred eight papers were found; of these, 22 were retained for this review. The treatment of HLH in adult is based on the HLH-94 regimen. The response rate is lower than in pediatric patients, and 20-30% are refractory to this therapy. DEP regimen and allogenic hematopoietic stem cell transplantation (HSCT) are associated with complete response and partial response in 27% and 49.2%, respectively. However, many patients fail to achieve a stable condition before HSCT, and mortality is higher in them. New drugs have been developed, such as emapalumab, ruxolitinib, and alemtuzumab, and they may be used as bridges to the curative HSCT. They are relatively well tolerated and have few or mild side effects. With these agents, the rate of partial response ranges from 14.2% to 100%, while the rate of complete response is highly variable according to study and medication used. The number of patients who achieved HSCT ranged from 44.8% to 77%, with a survival rate of 55.9% to 100%. However, the populations in these studies are mainly composed of mixed-age patients (pediatric and adult patients), and studies including only adult patients are scarce.
conclusionRelapsed or refractory HLH in adult patients is associated with poor outcome, and consolidation with HSCT may be required in some cases. Mortality related to HSCT is mainly due to active HLH disease before HSCT and post HSCT complications. New drugs, such as empalumab, ruxolitinib, and alemtuzumab are interesting since these agents may be used as bridges to HSCT with increases in the numbers of patients proceeding to HSCT and survival rate.
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