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Use of the JAK Inhibitor Ruxolitinib in the Treatment of Hemophagocytic Lymphohistiocytosis.
Keenan C, Nichols KE, Albeituni S. Keenan C, et al. Front Immunol. 2021 Feb 16;12:614704. doi: 10.3389/fimmu.2021.614704. eCollection 2021. Front Immunol. 2021. PMID: 33664745 Free PMC article. Review.
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome driven by overactive T cells and macrophages that abundantly secrete numerous pro-inflammatory cytokines, including interferon (IFN)-gamma, interleukin (IL)-1-beta, IL-2, IL-6, IL-10, IL-1 …
Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome driven by overactive T cells and macrophages tha …
Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults.
Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, Janka G, Jordan MB, van Laar JAM, Lachmann G, Lehmberg K, Machowicz R, Miettunen P, La Rosée P, Shakoory B, Zinter MS, Henter JI. Hines MR, et al. Crit Care Med. 2022 May 1;50(5):860-872. doi: 10.1097/CCM.0000000000005361. Epub 2021 Oct 5. Crit Care Med. 2022. PMID: 34605776
OBJECTIVE: Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome that often requires critical care support and remains difficult to diagnose. ...Early steroid treatment is indicated for patients with familial hemophagocytic lymph …
OBJECTIVE: Hemophagocytic lymphohistiocytosis is a hyperinflammatory syndrome that often requires critical care support …
A study of ruxolitinib response-based stratified treatment for pediatric hemophagocytic lymphohistiocytosis.
Zhang Q, Zhao YZ, Ma HH, Wang D, Cui L, Li WJ, Wei A, Wang CJ, Wang TY, Li ZG, Zhang R. Zhang Q, et al. Blood. 2022 Jun 16;139(24):3493-3504. doi: 10.1182/blood.2021014860. Blood. 2022. PMID: 35344583 Free article.
Hemophagocytic lymphohistiocytosis (HLH) is a lethal disorder characterized by hyperinflammation. ...Epstein-Barr virus (EBV)-HLH patients were most sensitive to ruxolitinib, with an ORR of 87.5% (58.3% in CR). After ruxolitinib therapy, 57.7% (30/52) …
Hemophagocytic lymphohistiocytosis (HLH) is a lethal disorder characterized by hyperinflammation. ...Epstein-Barr virus (EBV)- …
Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment.
Carter SJ, Tattersall RS, Ramanan AV. Carter SJ, et al. Rheumatology (Oxford). 2019 Jan 1;58(1):5-17. doi: 10.1093/rheumatology/key006. Rheumatology (Oxford). 2019. PMID: 29481673 Review.
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. ...These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including …
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critica …
Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study.
Bergsten E, Horne A, Aricó M, Astigarraga I, Egeler RM, Filipovich AH, Ishii E, Janka G, Ladisch S, Lehmberg K, McClain KL, Minkov M, Montgomery S, Nanduri V, Rosso D, Henter JI. Bergsten E, et al. Blood. 2017 Dec 21;130(25):2728-2738. doi: 10.1182/blood-2017-06-788349. Epub 2017 Sep 21. Blood. 2017. PMID: 28935695 Free PMC article.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic HLH (FHL) and secondary HLH. ...At median follow-up of 5.2 years, 230 of 369 patients (62%) were alive (5-year pSu, 61%; 56%-67 …
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome comprising familial/genetic H …
The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis.
Bami S, Vagrecha A, Soberman D, Badawi M, Cannone D, Lipton JM, Cron RQ, Levy CF. Bami S, et al. Pediatr Blood Cancer. 2020 Nov;67(11):e28581. doi: 10.1002/pbc.28581. Epub 2020 Jul 29. Pediatr Blood Cancer. 2020. PMID: 32725881
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) can be familial or secondary, which is often triggered by infection or malignancy. ...Infectious triggers were identified for four patients and two patients had malignancies. The average treatment duration …
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) can be familial or secondary, which is often triggered by infectio …
Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society.
Ehl S, Astigarraga I, von Bahr Greenwood T, Hines M, Horne A, Ishii E, Janka G, Jordan MB, La Rosée P, Lehmberg K, Machowicz R, Nichols KE, Sieni E, Wang Z, Henter JI. Ehl S, et al. J Allergy Clin Immunol Pract. 2018 Sep-Oct;6(5):1508-1517. doi: 10.1016/j.jaip.2018.05.031. Epub 2018 Jul 4. J Allergy Clin Immunol Pract. 2018. PMID: 30201097 Review.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome requiring aggressive immunosuppressive therapy. Following 2 large international studies mainly targeting pediatric patients with familial disease and patients wit …
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome requiring aggressive immunosuppressi …
Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP).
Sandler RD, Tattersall RS, Schoemans H, Greco R, Badoglio M, Labopin M, Alexander T, Kirgizov K, Rovira M, Saif M, Saccardi R, Delgado J, Peric Z, Koenecke C, Penack O, Basak G, Snowden JA. Sandler RD, et al. Front Immunol. 2020 Mar 31;11:524. doi: 10.3389/fimmu.2020.00524. eCollection 2020. Front Immunol. 2020. PMID: 32296434 Free PMC article. Review.
We report estimated rates of sHLH/MAS of 1.09% (95% CI = 0.89-1.30) following allogeneic HSCT, 0.15% (95% CI = 0.09-5.89) following autologous HSCT and 3.48% (95% CI = 0.95-6.01) following CAR-T cell therapy. ...Further research in this field is needed to raise awar …
We report estimated rates of sHLH/MAS of 1.09% (95% CI = 0.89-1.30) following allogeneic HSCT, 0.15% (95% CI = 0.09-5.89) following a …
Management of hemophagocytic lymphohistiocytosis in pregnancy: Case series study and literature review.
Liu C, Gao J, Liu J. Liu C, et al. J Obstet Gynaecol Res. 2022 Mar;48(3):610-620. doi: 10.1111/jog.15133. Epub 2022 Jan 2. J Obstet Gynaecol Res. 2022. PMID: 34978123 Free PMC article. Review.
AIM: The diagnosis and treatment of hemophagocytic lymphohistiocytosis (HLH) in pregnancy is challenging due to its rarity. ...Fetal or neonatal death up to 1 week after delivery occurred in eight (61.5%) pregnancies. CONCLUSIONS: Early diagnosis and treatmen …
AIM: The diagnosis and treatment of hemophagocytic lymphohistiocytosis (HLH) in pregnancy is challenging due to its rarity. .. …
Survival in primary hemophagocytic lymphohistiocytosis, 2016 to 2021: etoposide is better than its reputation.
Böhm S, Wustrau K, Pachlopnik Schmid J, Prader S, Ahlmann M, Yacobovich J, Beier R, Speckmann C, Behnisch W, Ifversen M, Jordan M, Marsh R, Naumann-Bartsch N, Mauz-Körholz C, Hönig M, Schulz A, Malinowska I, Hines M, Nichols KE, Gil-Herrera J, Talano JA, Crooks B, Formankova R, Jorch N, Bakhtiar S, Kühnle I, Streiter M, Nathrath M, Russo A, Dürken M, Lang P, Lindemans C, Henter JI, Lehmberg K, Ehl S. Böhm S, et al. Blood. 2024 Mar 7;143(10):872-881. doi: 10.1182/blood.2023022281. Blood. 2024. PMID: 37992218
Primary hemophagocytic lymphohistiocytosis (pHLH) is a life-threatening hyperinflammatory syndrome that develops mainly in patients with genetic disorders of lymphocyte cytotoxicity and X-linked lymphoproliferative syndromes. ...Importantly, early HSCT for patients …
Primary hemophagocytic lymphohistiocytosis (pHLH) is a life-threatening hyperinflammatory syndrome that develops mainly in pat …
80 results