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Journal articles on the topic 'HLH-criteria'

Author: Grafiati

Published: 7 June 2025

Last updated: 2 August 2025

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1

Smits, Bas M., Joris van Montfrans, Samuel A. Merrill, et al. "A Minimal Parameter Set Facilitating Early Decision-making in the Diagnosis of Hemophagocytic Lymphohistiocytosis." Journal of Clinical Immunology 41, no. 6 (2021): 1219–28. http://dx.doi.org/10.1007/s10875-021-01005-7.

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AbstractHemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune dysregulation syndrome characterized by uncontrolled immune cell activation. Timely diagnosis is important, since early treatment can improve survival rates. However, completing all assessments needed to reach ≥5 positive criteria out of the 8 HLH-2004 criteria can be time consuming and may delay timely initiation of treatment. Hence, we applied a data-driven approach to identify a minimal parameter set for early decision-making towards the initiation of HLH-specific treatment. We retrospectively evaluated 165 patien

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2

Dokmanovic, Lidija, Nada Krstovski, Jelena Lazic, et al. "Extreme hypertriglyceridemia in an infant with hemophagocytic lymphohistiocytosis and hydroxycobalamin deficiency." Srpski arhiv za celokupno lekarstvo 143, no. 11-12 (2015): 744–47. http://dx.doi.org/10.2298/sarh1512744d.

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Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, cytopenias, hepatosplenomegaly and hemophagocytosis. HLH may be primary or secondary to infection, autoimmune disease or malignancy. Hypertriglyceridemia is a common abnormality in HLH and one of the HLH-2004 diagnostic criteria. Case Outline. We present an infant with severe hypotonia and hypoproteinemic edema who also had extreme hypertriglyceridemia (21 mmol/l) and was diagnosed with HLH based on six of eight HLH- 2004 criteria (fever, hepatosplenomegaly, bicytopenia, hyper

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Croden, Jennifer, Jennifer Grossman, and Haowei Sun. "External Validation of the HLH-2004 Diagnostic Criteria and H-Score for Diagnosis of Hemophagocytic Lymphohistiocytosis in Adults." Blood 136, Supplement 1 (2020): 44–45. http://dx.doi.org/10.1182/blood-2020-138589.

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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome associated with multi-organ failure and death. Diagnosis in adult patients is currently based on the HLH-2004 diagnostic criteria; however, these criteria were developed for pediatric HLH and have not been formally validated in adults. An alternative diagnostic score, the H-score, was developed for adults with reactive HLH. There have been few external validation studies comparing the diagnostic accuracy between the HLH-2004 criteria and H-score, mostly in critically ill patients. In this external validation

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4

Rosado, Flavia G., Elizabeth B. Rinker, W. D. Plummer, et al. "The diagnosis of adult-onset haemophagocytic lymphohistiocytosis: lessons learned from a review of 29 cases of bone marrow haemophagocytosis in two large academic institutions." Journal of Clinical Pathology 69, no. 9 (2016): 805–9. http://dx.doi.org/10.1136/jclinpath-2015-203577.

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AimsHaemophagocytic lymphohistiocytosis (HLH) is divided into paediatric (primary) and adult (secondary) types. While paediatric-HLH has been extensively characterised, similar studies in adults are limited. This study aims to evaluate the significance of the HLH diagnostic criteria as well as other clinical parameters in adults with bone marrow evidence of haemophagocytosis.MethodsWe conducted a 10-year retrospective search of the pathology archives of two institutions for cases with bone marrow haemophagocytosis. We included those cases that fulfilled the currently established HLH diagnostic

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Meena, Nikhil K., Odai Sinokrot, Abhijit Duggal, et al. "The Performance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis in Critically Ill Patients." Journal of Intensive Care Medicine 35, no. 12 (2019): 1476–82. http://dx.doi.org/10.1177/0885066619837139.

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Objective: The diagnostic criteria for secondary hemophagocytic lymphohistiocytosis (HLH) have not been validated in the critically ill adult population. We set out to evaluate the performance of diagnostic criteria and determine the ferritin cutoff in critically ill adults. Design: A retrospective single-center study. Setting and Patients: Patients admitted to intensive care unit between 2008 and March 2010. Data were collected on consecutive patients who had ferritin measured. Charts were reviewed for the diagnostic criteria of HLH and components of Hscore. Measurements and Main Results: A t

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Park, Sunmin, Juwon Kim, Hyo Youl Kim, Young Uh, and Young Keun Kim. "Severe Fever with Thrombocytopenia Syndrome Virus Infection Associated with Hemophagocytic Lymphohistiocytosis as Poor Prognostic Factor." Open Forum Infectious Diseases 4, suppl_1 (2017): S319. http://dx.doi.org/10.1093/ofid/ofx163.749.

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Abstract Background Severe fever with thrombocytopenia (SFTS) is an emerging infectious disease caused by a novel bunyavirus designated SFTS virus (SFTSV) with a high fatality rate. Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated life-threatening disease triggered by infections, neoplasms and noninfectious inflammatory diseases. A few HLH associated with SFTSV were reported. According to the diagnostic criteria of HLH, 11 patients with SFTS were reviewed. Methods During last 2 years (2015–2016), 11 SFTS patients were diagnosed at the Wonju Severance Christian Hospital, Yonsei Un

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Ma, Alice D., Yuri D. Fedoriw, and Philip Roehrs. "Hyperferritinemia and Hemophagocytic Lymphohistiocytosis. Single Institution Experience in Adult and Pediatric Patients." Blood 120, no. 21 (2012): 2135. http://dx.doi.org/10.1182/blood.v120.21.2135.2135.

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Abstract Abstract 2135 Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disorder characterized by immune dysregulation and hypercytokinemia. Diagnostic criteria include a genetic mutation consistent with familial HLH or the presence of 5 of 8 defined clinical criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis, low/absent NK cell function, hyperferritinemia, and elevated soluble CD25). In pediatrics, a ferritin value of >10,000 mχγ/L has been reported to have 90% sensitivity and 96% specificity in defining the presence of HLH

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8

Rao, Nikith, Ritika Menon, and Matthew Burke. "A Case of Adult-Onset Still's Disease with Secondary Hemophagocytic Lymphohistiocytosis." International Archives of Internal Medicine 7, no. 1 (2025): 1–5. https://doi.org/10.23937/2643-4466/1710043.

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Adult-Onset Still’s Disease (AOSD) with Secondary Hemophagocytic Lymphohistiocytosis (HLH) is an autoimmune disorder that is most commonly a diagnosis of exclusion. AOSD is a known trigger of secondary HLH, amongst other inflammatory and infectious processes, making the association between the two already rare diseases even more intriguing. It commonly presents with non-specific symptoms like fevers, myalgias, night sweats, chills, paresthesias, and is associated with a relapsing-remitting salmon colored rash. Our patient is a young African American woman in her 20s who was diagnosed with AOSD

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Priyadarshini, Lipsa, Meenakshi Mohapatro, Priyadarshini Biswal, Silla Alisha Patro, Devidutta Ramani Ranjan Rout, and Simadri Swetapadma Roul. "Deciphering the clinical profile and diagnostic framework of hemophagocytic lymphohistiocytosis in a tertiary care setting." MGM Journal of Medical Sciences 11, no. 4 (2024): 595–602. https://doi.org/10.4103/mgmj.mgmj_321_24.

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Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory disorder marked by intense yet ineffective immune activation, resulting in hemophagocytosis, systemic inflammation, and multi-organ dysfunction. The diagnosis has traditionally depended on the 2004 HLH criteria, while the HScore, introduced in 2014, presents an alternative diagnostic approach. Materials and Methods: A 2-year study was conducted on patients referred to the hematology division for bone marrow evaluation. Patients with fever, cytopenia, and organomegaly underwent comprehensive biochemical

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10

Logan, Aaron C., Robert Su, Tracy I. George, Holbrook E. Kohrt, Bruno C. Medeiros, and Ash A. Alizadeh. "High Risk of Early Mortality in Adult Patients with Acquired Hemophagocytic Lymphohistiocytosis." Blood 114, no. 22 (2009): 1359. http://dx.doi.org/10.1182/blood.v114.22.1359.1359.

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Abstract Abstract 1359 Poster Board I-381 Background Hemophagocytic lymphohistiocytosis (HLH) is a rare condition in which dysregulation of innate immune effectors including natural killer (NK) cells and macrophages leads to destruction of hematopoietic elements. HLH is most frequently reported in association with malignancies or viral infections, with heritable deficiencies in cytotoxicity pathways predisposing children (and rarely adults) to HLH. Criteria for diagnosing HLH include: histological evidence of hemophagocytosis, two or more cytopenias, hyperferritinemia, hypofibrinogenemia or hy

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Kumar, Ashish, Eily Cournoyer, and Leonard Naymagon. "Inflamed—HLH, MAS, or something else?" Hematology 2023, no. 1 (2023): 31–36. http://dx.doi.org/10.1182/hematology.2023000463.

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Abstract Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive and maladaptive inflammation. Primary HLH is most frequently encountered in young children, and, without timely recognition and therapy, can lead to multiorgan failure and death. It is most often diagnosed using the HLH-2004 criteria and by identifying pathological mutations. However, the HLH-2004 criteria are not specific for HLH, and patients can easily fulfill these diagnostic criteria in other proinflammatory states in which HLH-therapy would not be indicated, including hematologic malignancies, infections, and rh

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Reid, J., R. Demianets, and S. Rezk. "Hemophagocytic Lymphohistiocytosis (HLH): A Clinical and Pathological Grading System Based on the Presence of Hemophagocytosis." American Journal of Clinical Pathology 162, Supplement_1 (2024): S81—S82. http://dx.doi.org/10.1093/ajcp/aqae129.181.

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Abstract Introduction/Objective Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of disorders resulting from uncontrolled immune activation leading to severe hyperinflammation. The syndrome can be primary (genetic) or secondary to infections, malignancies, or autoimmune diseases. The diagnosis of HLH is challenging due to its nonspecific clinical presentation and the overlap with other conditions. This study aims to establish a reliable diagnostic approach for HLH by evaluating the accuracy of bone marrow histomorphological criteria in diagnosing the disease. Methods/Case Repo

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Jankovic, Srdja, Marija Cazic, Dragana Janic, Jelena Lazic, Predrag Rodic, and Nada Krstovski. "Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis: A single-center, ten-year-long experience." Srpski arhiv za celokupno lekarstvo, no. 00 (2022): 119. http://dx.doi.org/10.2298/sarh221028119j.

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Introduction/Objective. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, splenomegaly and cytopenias. Diagnosis of HLH requires at least five of the eight criteria set by the Histiocyte Society and poses a significant challenge to physicians. HLH-2004 criteria include measurement of plasma levels of soluble receptor for interleukin-2 (sIL-2R), an invaluable tool in the diagnosis of HLH, particularly because it can be measured swiftly and inexpensively. Methods. We retrospectively analyzed medical records of 45 pediatric patients (28 boys

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Fazal, Farhan, Naveet Wig, Manish Soneja, et al. "1011. Sepsis and Secondary Hemophagocytic Lymphohistiocytosis." Open Forum Infectious Diseases 5, suppl_1 (2018): S301. http://dx.doi.org/10.1093/ofid/ofy210.848.

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Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition diagnosed by HLH 2004 criteria. This criterion has common clinical and laboratory features with sepsis and tropical fevers, but there is marked difference in management and outcome of these two entities. The study is conducted to know whether there is any difference in the clinico-laboratory features, management, and outcome of sepsis with or without secondary HLH. Methods This is a prospective observational study where patients presenting with sepsis and bicytopenia are included. The

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Zoref Lorenz, Adi, Liron Hofstetter, Jun Murakami, et al. "Serum soluble CD25 and ferritin in distinguishing patients with uncomplicated hematologic malignancies from patients with hematologic malignancies complicated by hemophagocytic lymphohistiocytosis." Journal of Clinical Oncology 38, no. 15_suppl (2020): e20072-e20072. http://dx.doi.org/10.1200/jco.2020.38.15_suppl.e20072.

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e20072 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyper-inflammatory syndrome with distinct clinical and laboratory features. In adults, HLH is often associated with underlying malignancy, most commonly hematologic malignancies (HM). HLH occurs in 1% of adults with HM and overall survival can be low as 10-20%. Abnormal serum levels of the inflammatory markers sCD25 and ferritin are diagnostic criteria for familial HLH. However, because these reactants are often elevated in malignancy, appropriate levels for diagnosis in HM-HLH are unknown. In this study, we esta

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Leão, Inês, Maria Eduarda Couto, Joana Catarina Lima Marinho, Isabel Oliveira, Ângelo Martins, and Mário Mariz. "Adult malignancy-associated hemophagocytic lymphohistiocytosis: Case series." Journal of Clinical Oncology 39, no. 15_suppl (2021): e19579-e19579. http://dx.doi.org/10.1200/jco.2021.39.15_suppl.e19579.

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e19579 Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening hyperinflammatory syndrome, with a challenging diagnosis (pediatric HLH-2004 criteria) and treatment (HLH-1994 protocol). Recently, HScore has adapted these diagnostic criteria to the adult population. Malignancy-associated HLH (M-HLH) can occur as the first manifestation of an unknown malignancy or after / during the treatment of a known cancer. This study evaluates features and outcomes of M-HLH in adult patients. Methods: Retrospective cohort study of all patients diagnosed with M-HLH in a single inst

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Ismail, Ahmad Khaldun. "Haemophagocytic Lymphohistiocytosis (HLH) in Adult with Dengue Infection." Medicine & Health 16, no. 2 (2021): 287–94. http://dx.doi.org/10.17576/mh.2021.1602.23.

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Haemophagocytic lymphohistiocytosis (HLH) or haemophagocytic syndrome is a rare but life-threatening syndrome of excessive immune activation with nonspecific clinical presentation. HLH is one of the complications in dengue infection. A 69-year-old lady was treated for severe dengue with multi-organ dysfunction with superimposed pneumonia, requiring mechanical ventilation. However, persistent cytopenia despite blood transfusion without evidence of haemorrhage raised the suspicion of HLH. Further blood investigations revealed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia. Bon

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Zoref-Lorenz, Adi, Jun Murakami, Liron Hofstetter, et al. "An improved index for diagnosis and mortality prediction in malignancy-associated hemophagocytic lymphohistiocytosis." Blood 139, no. 7 (2022): 1098–110. http://dx.doi.org/10.1182/blood.2021012764.

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Abstract Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome that may complicate hematologic malignancies (HMs). The appropriateness of current criteria for diagnosing HLH in the context of HMs is unknown because they were developed for children with familial HLH (HLH-2004) or derived from adult patient cohorts in which HMs were underrepresented (HScore). Moreover, many features of these criteria may directly reflect the underlying HM rather than an abnormal inflammatory state. To improve and potentially simplify HLH diagnosis in patients with HMs, we studied a

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Janković, Srđa, Marija Ćazić, Dragana Janić, Jelena Lazić, Predrag Rodić, and Nada Krstovski. "Soluble interleukin-2 receptor in pediatric patients investigated for hemophagocytic lymphohistiocytosis – a single-center, 10-year-long experience." Srpski Arhiv Celokupno Lekarstvo 151, no. 1-2 (2022): 43–49. https://doi.org/10.2298/SARH221028119J.

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SUMMARY Introduction/Objective Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition characterized by fever, splenomegaly, and cytopenias. Diagnosis of HLH requires at least five of the eight criteria set by the Histiocyte Society and poses a significant challenge to physicians. HLH-2004 criteria include measurement of plasma levels of soluble receptor for interleukin-2 (sIL-2R), an invaluable tool in the diagnosis of HLH, particularly because it can be measured swiftly and inexpensively. Methods We retrospectively analyzed medical records of 45 pediatric patients (2

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Pham, Anthony Q., Megan M. O'Byrne, Prashant Kapoor, et al. "Analysis of Serum Ferritin Levels As a Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis (HLH) in Hospitalized Adult Patients." Blood 126, no. 23 (2015): 1014. http://dx.doi.org/10.1182/blood.v126.23.1014.1014.

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Abstract INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder caused by the pathologic activation of the immune system. In children, either a molecular diagnosis consistent with HLH or five out of the following eight criteria are considered necessary for a diagnosis of HLH (HLH-04 criteria): 1) fever; 2) splenomegaly; 3) cytopenia in two or more cell lines; 4) hypertriglyceridemia (≥265 mg/dL) or hypofibrinogenemia (≤150 mg/dL); 5) hemophagocytosis in the bone marrow, spleen, or lymph nodes; 6) hyperferritinemia (≥500 mcg/L); 7) impaired NK cell function; and 8) elevated s

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Otrock, Zaher K., Alexandra Wolanskyj, Charles S. Eby, Roshini Abraham, Prashant Kapoor, and Sameer A. Parikh. "Bi-Institutional Study of Etiology, Clinical Characteristics and Outcomes of Adult Patients with Hemophagocytic Lymphohistiocytosis." Blood 124, no. 21 (2014): 2724. http://dx.doi.org/10.1182/blood.v124.21.2724.2724.

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Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe clinical syndrome characterized by the activation of mononuclear phagocytic system. Patients (pts) typically present with fever, cytopenias, splenomegaly, hyperferritinemia and hemophagocytosis. There are two categories of HLH: primary (familial) and secondary (acquired). Primary HLH (FHL) is caused by genetic defects in proteins involved in cytotoxic function in T and NK cells, and occurs most commonly in children; though hypomorphic mutations in the same genes may cause adult-onset FHL. In contrast, secondary HLH typic

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Taieb, Guillaume, Elsa Kaphan, Claire Duflos, et al. "Hemophagocytic Lymphohistiocytosis Gene Mutations in Adult Patients Presenting With CLIPPERS-Like Syndrome." Neurology - Neuroimmunology Neuroinflammation 8, no. 3 (2021): e970. http://dx.doi.org/10.1212/nxi.0000000000000970.

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ObjectiveTo determine whether adult cases of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) may be related to familial hemophagocytic lymphohistiocytosis (HLH) causes, we have screened patients with adult-onset CLIPPERS for mutations in primary HLH-associated genes.MethodsIn our cohort of 36 patients fulfilling the criteria for probable or definite CLIPPERS according to the CLIPPERS-2017 criteria, we conducted a first study on 12 patients who consented to genetic testing. In these 12 patients, systemic HLH criteria were searched, and ge

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Lachmann, Gunnar, Cornelia Knaak, Clarissa von Haefen, et al. "Diagnostic biomarkers for adult haemophagocytic lymphohistiocytosis in critically ill patients (HEMICU): a prospective observational study protocol." BMJ Open 9, no. 10 (2019): e032695. http://dx.doi.org/10.1136/bmjopen-2019-032695.

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IntroductionHaemophagocytic lymphohistiocytosis (HLH) in adults is characterised by toxic immune activation and a sepsis-like syndrome, leading to high numbers of undiagnosed cases and mortality rates of up to 68%. Early diagnosis and specific immune suppressive treatment are mandatory to avoid fatal outcome, but the diagnostic criteria (HLH-2004) are adopted from paediatric HLH and have not been validated in adults. Experimental studies suggest biomarkers to sufficiently diagnose HLH. However, biomarkers for the diagnosis of adult HLH have not yet been investigated.Methods and analysisThe HEM

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Raj, Swati. "Peripheral hemophagocytosis- A key to decifer hemophagocytic lymphohistiocytosis, an extremely rare case report." IP Journal of Diagnostic Pathology and Oncology 9, no. 4 (2024): 224–29. https://doi.org/10.18231/j.jdpo.2024.046.

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Hemophagocytic lymphohistiocytosis(HLH) is an uncommon and probably underdiagnosed disorder that requires quick medical attention because of its high and rapid mortality rate. The present criteria are the only ones used to diagnose HLH. For a laboratory diagnosis of HLH-2007(an upgraded version of HLH-2004), five of the eight requirements must be satisfied. We report an exceedingly rare case of a thirty-year-old Indian man who has been clinically presenting with a high-grade fever, weakness, oligouria, and speech slurring for 20 days. While screening peripheral blood smear(PBS) in order to cor

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Meyer, Lauren K., Erica A. Steen, Camille Keenan, Adam B. Olshen, Kim E. Nichols, and Matt S. Zinter. "A Real-World Analysis of the Diagnostic Evaluation for Hemophagocytic Lymphohistiocytosis in a Pediatric Population." Blood 142, Supplement 1 (2023): 3919. http://dx.doi.org/10.1182/blood-2023-180086.

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Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome that is often fatal without immunosuppressive treatment to quell the overactive immune response. Accordingly, it is crucial to diagnose HLH promptly and accurately. Nevertheless, diagnosing HLH is challenging due to its phenotypic overlap with other inflammatory conditions. Furthermore, existing HLH diagnostic criteria consist largely of nonspecific clinical and laboratory findings. Together, these factors contribute to significant diagnostic uncertainty, which can delay initiation of treatment in patients who actu

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Hur, Joon Young, Kang Kook Lee, Jun Ho Jang, et al. "Clinical Relevance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis (HLH) and Survival Outcome of Adult HLH Patients: A Single-Center Prospective Cohort Study." Blood 132, Supplement 1 (2018): 3718. http://dx.doi.org/10.1182/blood-2018-99-114858.

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Abstract Introduction Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated life-threatening condition in which activated macrophages phagocytize hematopoietic cells in various clinical situations from physiologic reactions to pathologic conditions such as malignancy. The diagnosis of HLH is currently done according to the HLH-2004 criteria which were mainly based on pediatric experiences even though underlying causes and clinical features of adult patients with HLH are different from pediatric patients. However, there is little information about the value of each item and its clinica

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Upadhyay Banskota, Shristi, Andres E. Mendez-Hernandez, Hafeez Shaka, et al. "Adult Hemophagocytic Lymphohistiocytosis(HLH): Experience of an Urban, Public Hospital over Two Decades." Blood 136, Supplement 1 (2020): 19. http://dx.doi.org/10.1182/blood-2020-141947.

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Introduction:HLH is a rare, life-threatening disorder, characterized by hyperstimulation of immune system leading to systemic inflammation and multi-organ failure. It is categorized as primary and secondary HLH. Secondary HLH usually affects adolescents and adults. It results from acquired immune dysregulation secondary to a number of etiologies, including infections, malignancy, and autoimmune diseases. Owing to less epidemiological data, adult HLH is thought to be underdiagnosed, making a true assessment difficult, however, some observational data suggest 40% of HLH cases occurs in adults. D

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Chu, Raymond, Charmaine van Eeden, Sneha Suresh, Wendy I. Sligl, Mohammed Osman, and Jan Willem Cohen Tervaert. "Do COVID-19 Infections Result in a Different Form of Secondary Hemophagocytic Lymphohistiocytosis." International Journal of Molecular Sciences 22, no. 6 (2021): 2967. http://dx.doi.org/10.3390/ijms22062967.

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The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has resulted in significant morbidity and mortality across the world, with no current effective treatments available. Recent studies suggest the possibility of a cytokine storm associated with severe COVID-19, similar to the biochemical profile seen in hemophagocytic lymphohistiocytosis (HLH), raising the question of possible benefits that could be derived from targeted immunosuppression in severe COVID-19 patients. We reviewed the literature regarding the diagnosis and fea

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Wulyansari, Wulyansari, and Yetti Hernaningsih. "Laboratory Examination in Hemophagocytic Lymphohistiocytosis." INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY 28, no. 1 (2021): 84–90. http://dx.doi.org/10.24293/ijcpml.v28i1.1881.

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Hemophagocytic Lymphohistiocytosis (HLH) is derived from the word hemophagocytosis, in which macrophagesinfiltrate tissue extensively, and unspecifically phagocyte blood and bone marrow cells. The deviant activation of cytotoxicCD8+ T-cells causing the release of inflammatory cytokines is the core pathogenesis of HLH. Hemophagocyticlymphohistiocytosis is a regulatory disorder of the immune system, with clinical signs and symptoms of extremeinflammation and cytopenia, hepatitis, and severe and life-threatening central nervous system dysfunction. The name of theHLH disorder was recently proposed

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Suman, Saurav, and Aleksander Lenert. "Diagnostic Dilemma of Disseminated Histoplasmosis Mimicking Hemophagocytosis Lymphohistiocytosis in Patient with Rheumatoid Arthritis on Anti-TNF Therapy: Case Report and Review of the Literature." Case Reports in Rheumatology 2019 (February 12, 2019): 1–3. http://dx.doi.org/10.1155/2019/4169052.

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Tumor necrosis factor inhibitors (TNFi) have become the cornerstone for the treatment of rheumatoid arthritis and other systemic autoimmune conditions. However, these biologic DMARDs can lead to various opportunistic infections such as viral infection, tuberculosis, and histoplasmosis. Furthermore, these biologics can also cause severe systemic inflammatory reactions known as hemophagocytosis lymphohistiocytosis (HLH) that can lead to multiorgan failure and high mortality. Due to overlapping clinical features and time-intensive microbiological culture methods, distinguishing between HLH and op

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Cervoni-Curet, Frances Natalia, Adan Rios, Binoy Yohannan, and Hongyu Miao. "Secondary hemophagocytic lymphohistiocytosis (HLH): Five years’ experience at the UTHealth McGovern Medical School at Houston, Texas." Journal of Clinical Oncology 39, no. 15_suppl (2021): e19578-e19578. http://dx.doi.org/10.1200/jco.2021.39.15_suppl.e19578.

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e19578 Background: Secondary HLH in adults is associated to infections, malignancies, and autoimmune disorders. HLH in children has been the basis for the management and treatment of HLH in adults. Despite their clinical similarities there are fundamental differences. Children’s HLH is caused by gene mutations in granule-mediated cytotoxicity while secondary HLH does not have known apparent genetic causes. This may affect the clinical outcomes based in how we approach the diagnosis and management of secondary HLH in adults. Methods: We reviewed 49 cases of secondary HLH at our institution over

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Anabtawi, Amir, Reem Alkilany, and Mary E. Lacy. "Hemophagocytic Lymphohistiocytosis in a Patient With Advanced HIV and Cytomegalovirus Infection." Journal of Investigative Medicine High Impact Case Reports 8 (January 2020): 232470962090696. http://dx.doi.org/10.1177/2324709620906961.

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive, and, if not treated, fatal disorder that is characterized by excessive immune system activation. This disorder can be precipitated by different triggers including malignancies, infections, and autoimmune disorders. Diagnosis is made by fulfilling criteria that was last updated in 2004, and treatment frequently includes management of the underlying trigger but can also include chemotherapy. In this article, we report a case of HLH in a 27-year-old male, who had been diagnosed with advanced untreated HIV, who presented to the hospit

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Ganesh, Vijay, Aneesh Mohimen, Puneet Singh, and Sanjeev Khera. "Isolated central nervous system haemophagocytic lymphohistiocytosis with PRF1 gene mutation presenting as fever of unknown origin." BMJ Case Reports 18, no. 2 (2025): e262752. https://doi.org/10.1136/bcr-2024-262752.

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Central nervous system (CNS) manifestations are seen in two-thirds cases of familial haemophagocytic lymphohistiocytosis (HLH). Isolated CNS-HLH is described as a rare entity characterised by isolated neuroinflammation without fulfilling diagnostic criteria for evidence of systemic inflammation in mutation-proven familial HLH due to additional genetic modifiers. We describe one such female preschooler who presented to us with the fever of unknown origin spanning over a year and was a diagnostic dilemma. Only two out of the available seven criteria were fulfilled for systemic HLH in the index c

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Tremblay-Lemay, Rosemarie, Lourdes Calvente, Monali Gupta, et al. "S100B Is a Useful Marker for the Diagnosis of Haemophagocytic Lymphohistiocytosis." Blood 132, Supplement 1 (2018): 3711. http://dx.doi.org/10.1182/blood-2018-99-116783.

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Abstract Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening systemic inflammatory clinical syndrome that can be primary/familial or secondary to a variety of underlying conditions. The 2004 diagnostic criteria for HLH require 5 out of 8 of the following clinical and pathological variables to be present: fever, splenomegaly, cytopenia in at least 2 lineages, hypertriglyceridemia/hypofibrinogenemia, haemophagocytosis on pathology examination, low/absent NK cell activity, ferritin greater than 500µg/L or soluble IL-2 receptor (sCD25) greater than 2400 U/mL. At least 5 of these criter

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Gualdoni, Hofmann, Wohlfarth, et al. "Prevalence and Outcome of Secondary Hemophagocytic Lymphohistiocytosis Among SIRS Patients: Results from a Prospective Cohort Study." Journal of Clinical Medicine 8, no. 4 (2019): 541. http://dx.doi.org/10.3390/jcm8040541.

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Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening condition clinically presenting as SIRS (Systemic Inflammatory Response Syndrome). However, there is no comprehensive data concerning diagnostic algorithms, prevalence, outcome and biomarker performance in SIRS patients. We conducted a prospective observational cohort study on 451 consecutive patients fulfilling ≥2 SIRS criteria. The Hscore and the HLH-2004 criteria were used to determine the presence of sHLH, and the correlation of the screening-biomarkers ferritin, sCD25, and sCD163 with both scores was assessed. Out o

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Lenz, Max, Patrick Haider, Eva Steinacher, et al. "Markers of Hemophagocytic Lymphohistiocytosis Are Associated with Mortality in Critically Ill Patients." Journal of Clinical Medicine 14, no. 6 (2025): 1970. https://doi.org/10.3390/jcm14061970.

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Background: Critically ill patients often display systemic immune dysregulation and increased inflammatory activity. Hemophagocytic lymphohistiocytosis (HLH) represents a rare syndrome defined by the inappropriate survival of cytotoxic T cells and the occurrence of cytokine storms. Although HLH is characterized by relatively high mortality rates, little is known about the predictive value of its diagnostic criteria. Accordingly, our objective was to evaluate these properties within an unselected cohort of critically ill patients admitted to a tertiary intensive care unit (ICU). Methods: This s

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Dziedzic, Radosław, Stanisława Bazan-Socha, Mariusz Korkosz, and Joanna Kosałka-Węgiel. "Characteristics of 21 Patients with Secondary Hemophagocytic Lymphohistiocytosis—Insights from a Single-Center Retrospective Study." Medicina 61, no. 6 (2025): 977. https://doi.org/10.3390/medicina61060977.

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Background and Objectives: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition characterized by excessive activation of cytotoxic lymphocytes and macrophages, resulting in a cytokine storm, multiorgan damage, and high mortality. HLH is classified into primary (genetic) and secondary (acquired) forms, with diagnosis often challenging due to nonspecific symptoms. Macrophage activation syndrome (MAS) refers to the secondary HLH triggered by rheumatic diseases. In this study, we retrospectively analyzed the clinical and laboratory features of patients with secondary HLH

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Kim, Dong Won, Forat Lutfi, Facundo Zafforoni, et al. "Low Utility of the H-Score and HLH-2004 Criteria to Identify Patients with Secondary Hemophagocytic Lymphohistiocytosis after CAR-T Cell Therapy for Relapsed/Refractory Diffuse Large B-Cell Lymphoma." Blood 136, Supplement 1 (2020): 3–4. http://dx.doi.org/10.1182/blood-2020-141992.

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Introduction: Secondary hemophagocytic lymphohistiocytosis (HLH) is an aggressive life-threatening activation of the immune system triggered by an underlying condition. Use of chimeric antigen receptor therapy (CAR-T) to treat relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL) is associated with cytokine release syndrome (CRS), Immune Effector Cell Associated Neurotoxicity Syndrome (ICANS) and development of secondary HLH. Application of HLH scoring systems such as the H-score or HLH-2004 criteria to identify CAR-T triggered HLH is not validated in this setting. Inability to promptl

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Alekseeva, Ekaterina I., Rustem F. Tepaev, Ilia Y. Shilkrot, Tatyana M. Dvoryakovskaya, Aleksander G. Surkov, and Ivan A. Kriulin. "COVID-19-associated secondary hemophagocytic lymphohistiocytosis (cytokine storm syndrome)." Annals of the Russian academy of medical sciences 76, no. 1 (2021): 51–66. http://dx.doi.org/10.15690/vramn1410.

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In most cases, COVID-19 has a favorable outcome. However, the risk of developing critical forms of the disease, including secondary hemophagocytic lymphohistiocytosis HLH (cytokine storm syndrome), remains high. This dictates the interest in studying pathogenetic mechanisms, features of the clinical picture, laboratory and instrumental criteria for covid-19 disease. The article analyzes the causes of acute respiratory distress syndrome and multiple organ failure as manifestations of HLH. The necessity of monitoring signs of hyperinflammation (ferritin, C-reactive protein, etc., biomarkers of i

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Alsaedi, Hawazen S., Michael Stump, Wenzinger Christopher, Lazarchick John, and Jennifer Jaroscak. "Pediatric Hemophagocytic Lymphohistocytosis (HLH): A Single Institution Experience in the Diagnosis and Management of This Variable Disease." Blood 124, no. 21 (2014): 4982. http://dx.doi.org/10.1182/blood.v124.21.4982.4982.

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Abstract Background: Hemophagocytic Lymphohistocytosis (HLH) is a rare disease that can be fatal if left untreated. We present a single institution series of pediatric patients diagnosed with HLH. Infants and children can present acutely ill with a febrile illness, and a high index of suspicion is required to make the diagnosis. Diagnosing HLH is challenging due to the rare occurrence, variable presentation, and nonspecific findings of this disorder. We report on 17 patients diagnosed with HLH in this series. Methods: This is a single institution review of pediatric cases of HLH diagnosed from

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Ellis, Esther M., Janice Pérez-Padilla, Liza González, et al. "Unusual Cluster In Time and Space Of Dengue-Associated Hemophagocytic Lymphohistiocytosis In Puerto Rico." Blood 122, no. 21 (2013): 3497. http://dx.doi.org/10.1182/blood.v122.21.3497.3497.

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Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal hematologic syndrome characterized by hyperinflammation due to uncontrolled proliferation of activated lymphocytes, resulting in prolonged fever, pancytopenia, jaundice, and hepatosplenomegaly. While infection with Epstein Barr virus (EBV) is the most common recognized cause of infection-triggered HLH, there have been cases associated with other viral infections including dengue. Dengue is an acute febrile illness that is caused by any of four related but serologically distinct, mosquito-borne dengue viruses (DENV-1

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Oh, Eun-Jee, Jae-Ho Yoon, Ki Hyun Park, et al. "Natural-killer cell cytotoxicity as a diagnostic and prognostic marker for adult patients with secondary hemophagocytic lymphohistiocytosis: a prospective phase II observational study." Therapeutic Advances in Hematology 12 (January 2021): 204062072110205. http://dx.doi.org/10.1177/20406207211020544.

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Background: Hemophagocytic lymphohistiocytosis (HLH) can be life-threatening if not detected and treated appropriately. The diagnosis of HLH can be confusing due to other similar febrile diseases that present with cytopenia. Natural-killer cell (NK)-cytotoxicity is an important diagnostic parameter for primary HLH; however, its role in secondary HLH in adults has not been well-elucidated. Methods: We prospectively enrolled 123 adult patients with febrile conditions accompanied by cytopenia or marrow hemophagocytosis. A diagnosis of HLH was based on HLH-2004 criteria and treated based on HLH-94

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Zoref Lorenz, Adi, Jun Murakami, Liron Hofstetter, et al. "A novel index using inflammatory markers improves the diagnosis of hemophagocytic lymphohistiocytosis in patients with hematologic malignancies." Journal of Clinical Oncology 39, no. 15_suppl (2021): 7563. http://dx.doi.org/10.1200/jco.2021.39.15_suppl.7563.

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7563 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome that may accompany hematologic malignancies (HM). The diagnosis of HLH in patients with HM (HM-HLH) is confounded by a number of factors: the most commonly used HLH-2004 diagnostic criteria are derived from studies in infants while the Hscore used in adults is not specific for HMs; moreover, most parameters in these scoring systems may reflect features of the underlying HM rather than HLH associated inflammation; and finally specific diagnostic cutoff values for laboratory abnormalities in HM-

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Nielsen, Tine, Andreja Dimitrijevic, Mia Dahl Sørensen, Isik Somuncu Johansen, and Dennis Lund Hansen. "Importance of immunosuppression in haemophagocytic lymphohistiocytosis caused by miliary tuberculosis." BMJ Case Reports 17, no. 9 (2024): e261936. http://dx.doi.org/10.1136/bcr-2024-261936.

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Haemophagocytic lymphohistiocytosis (HLH) is a syndrome with an abnormal activation of the immune system and is associated with a high mortality even with treatment. We present a case of a woman in her mid-50s who developed HLH triggered by miliary tuberculosis (TB) while receiving a tumour necrosis factor alpha inhibitor.The patient was admitted with a high fever and respiratory pain. Her condition deteriorated despite empirical treatment. Diagnosis of HLH was established based on clinical presentation, H-score and HLH-04 criteria. Concurrently, miliary TB was identified as the trigger. She w

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Trivedi, Krunal, Hamid Shaaban, Stephen Abo, and Nibras yar Khan. "HLH Secondary to Influenza." Blood 142, Supplement 1 (2023): 5369. http://dx.doi.org/10.1182/blood-2023-184905.

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Introduction Hemophagocytic lymphohistiocytosis (HLH) is a pathological condition characterized by excessive inflammation and tissue damage resulting from an aberrant immune system response. HLH can be classified into two distinct categories: primary HLH and secondary HLH. Secondary hemophagocytic lymphohistiocytosis (HLH) is an acquired condition that can be precipitated by various infections, malignancies, or rheumatological disorders. Herein, we present a rare case of HLH secondary to influenza B infection. Case Presentation A 37-year-old who routinely vapes and smokes cannabis presented to

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Schram, Alison M., Ann Mullally, Annemarie E. Fogerty, Elena Massarotti, and Nancy Berliner. "Hemophagocytic Lymphohistiocytosis: The Partners Healthcare Experience over the Past 8 Years." Blood 124, no. 21 (2014): 4104. http://dx.doi.org/10.1182/blood.v124.21.4104.4104.

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Abstract Introduction Hemophagocytic Lymphohistiocytosis (HLH) is a rare disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of severe inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Familial HLH typically presents in childhood and is well described. Less is known about the clinical characteristics, appropriate diagnostic criteria, and optimal treatment in the adult population. The purpose of this study

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Birndt, Sebastian, Thomas Schenk, Frank M. Brunkhorst, et al. "Hemophagocytic Lymphohistiocytosis in Adults (aHLH): Results from the German HLH Registry." Blood 128, no. 22 (2016): 2523. http://dx.doi.org/10.1182/blood.v128.22.2523.2523.

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Abstract Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome caused by excessive immune activation leading to a life-threatening pro-inflammatory cytokine storm. HLH is not an entity of its own, but a clinical syndrome triggered by various conditions like infections, malignancy or autoimmune disorders. HLH pathogenesis is complex and still not fully understood. Contributing factors include immunosuppression (chemotherapy, long-term immunosuppressive therapy), cytokine release from tumor cells, imbalance between infected and immune effector cells as wel

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Takahashi, Takashi, and Jeffrey Rykken. "Progressive Diffuse Osteonecrosis in a Patient with Secondary Hemophagocytic Lymphohistiocytosis." Case Reports in Radiology 2015 (2015): 1–6. http://dx.doi.org/10.1155/2015/730719.

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This is a case report with serial imaging showing progression of diffuse osteonecrosis in a patient after a diagnosis of secondary hemophagocytic lymphohistiocytosis (HLH). While bone marrow involvement in HLH has been long noted at histological evaluation and is itself one of the diagnosis criteria, to the best of our knowledge, there has been no previous publication addressing osseous image findings in a patient with HLH.

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Janssen, Jesslyn, Chiamaka Nwachukwu, and Corey Paul Falcon. "Post-COVID-19 Infant with a Hemizygous PRF1 Mutation and Overlapping Features between MIS-C and Hemophagocytic Lymphohistiocytosis." Blood 144, Supplement 1 (2024): 5377. https://doi.org/10.1182/blood-2024-209294.

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Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disorder that requires a high index of suspicion and comprehensive evaluation for timely recognition. Its rarity, diverse clinical manifestations, and overlapping symptoms with other hyperinflammatory syndromes, such as the recently emerged Multisystem Inflammatory Syndrome in Children (MIS-C) during the COVID-19 pandemic, present unique diagnostic challenges. Herein, we report the case of an 8-month-old infant initially suspected of MIS-C following acute liver failure after COVID-19 exposure. The diagnosis was later revis

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Mohamed, Said Yousuf, Muhamad Hitham Almahayni, Wahiba Chebbo, et al. "Monocytopenia Is Universally Present in Adults with Hemophagocytic Syndrome/Hemophagocytic Lymphohistiocytosis (HLH), Responds to Treatment, and Can be Used As a Sensitive Additional Diagnostic Criterion and As a Prognostic Marker." Blood 128, no. 22 (2016): 1330. http://dx.doi.org/10.1182/blood.v128.22.1330.1330.

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Abstract Background HLH is an under-estimated and under-reported syndrome with diverse etiologies but can cause life threatening cytopenia (s) and organ failure with very high mortality in critically ill patients. Early diagnosis and management are of paramount importance for better survival. The current HLH 2004 diagnostic criteria are not optimal to achieve such a goal. Monocytopenia is not included in the diagnostic criteria although HLH is associated with activation, mobilization of and shift of monocytes to tissues where phagocytosis occurs. Hypothesis and Goal We hypothesized that monocy

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