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Relevant bibliographies by topics / HLH-criteria

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Academic literature on the topic 'HLH-criteria'

Author: Grafiati

Published: 7 June 2025

Last updated: 16 July 2025

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Journal articles on the topic "HLH-criteria"

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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3

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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5

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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6

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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7

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

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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9

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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10

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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