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JYMS : Journal of Yeungnam Medical Science

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Hyun Ji Lim 2 Articles
A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults
Abraham Kwak, Nani Jung, Ye Jee Shim, Heung Sik Kim, Hyun Ji Lim, Jae Min Lee, Mi Hwa Heo, Young Rok Do
Yeungnam Univ J Med. 2021;38(3):208-218.   Published online November 27, 2020
DOI: https://doi.org/10.12701/yujm.2020.00591
  • 6,037 View
  • 144 Download
  • 4 Crossref
AbstractAbstract PDF
Background
Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH.
Methods
The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated.
Results
Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1–83 years), and the median follow-up duration was 8.5 months (range, 0–204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH.
Conclusion
Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.

Citations

Citations to this article as recorded by  
  • Spontaneous resolution of hemophagocytic lymphohistiocytosis in a child infected with epstein–Barr virus
    Rita Alfattal, Hussain Sadeq, Abdullah Ali
    Journal of Applied Hematology.2023; 14(1): 57.     CrossRef
  • Pediatric inborn errors of immunity causing hemophagocytic lymphohistiocytosis: Case report and review of the literature
    María Soledad Caldirola, Andrea Gómez Raccio, Daniela Di Giovanni, María Isabel Gaillard, María Victoria Preciado
    Journal of Leukocyte Biology.2022; 112(4): 607.     CrossRef
  • Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
    Bruno Fattizzo, Marta Ferraresi, Juri Giannotta, Wilma Barcellini
    Journal of Clinical Medicine.2021; 10(4): 870.     CrossRef
  • HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient
    Lauren T. Maloney, Bronwyn Baz, Dia Hazra
    Pediatrics.2021;[Epub]     CrossRef
Delayed treatment-free response after romiplostim discontinuation in pediatric chronic immune thrombocytopenia
Hyun Ji Lim, Young Tae Lim, Jeong Ok Hah, Jae Min Lee
Yeungnam Univ J Med. 2021;38(2):165-168.   Published online August 7, 2020
DOI: https://doi.org/10.12701/yujm.2020.00493
  • 5,011 View
  • 136 Download
  • 3 Crossref
AbstractAbstract PDF
We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/μL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/μL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/μL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.

Citations

Citations to this article as recorded by  
  • A cost–utility analysis of thrombopoietin receptor agonists for treating pediatric immune thrombocytopenia purpura after failure of first‐line therapies
    Huimin Du, Jiamin Wang, Joel Livingston, Ziyad Alrajhi, Melanie Kirby‐Allen, Brian Chan, Rebecca Hancock‐Howard, Peter C. Coyte
    Pediatric Blood & Cancer.2023;[Epub]     CrossRef
  • Generic romiplostim for children with persistent or chronic immune thrombocytopenia: Experience from a tertiary care centre in North India
    Chandana Mareddy, Manas Kalra, Anupam Sachdeva
    British Journal of Haematology.2022; 197(5): 618.     CrossRef
  • Tapering of the thrombopoietin receptor agonist in paediatric patients with chronic immune thrombocytopenia: Is it possible?
    María Solsona, Rubén Berrueco, Elena Sebastián, Áurea Cervera, Ana Sastre, Itziar Astigarraga, Bienvenida Argilés, María Ángeles Dasí, José Luís Dapena, Emilio Monteagudo
    British Journal of Clinical Pharmacology.2022; 88(9): 4220.     CrossRef

JYMS : Journal of Yeungnam Medical Science