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JYMS : Journal of Yeungnam Medical Science

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3 "Hemophagocytic lymphohistiocytosis"
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Original article
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
  • 6,813 View
  • 148 Download
  • 6 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  
  • Prevalence and mortality of haemophagocytic lymphohistiocytosis in dengue fever: a systematic review and meta-analysis
    Leong Tung Ong, Roovam Balasubramaniam
    Transactions of The Royal Society of Tropical Medicine and Hygiene.2024;[Epub]     CrossRef
  • Predicting relapsed/refractory disease in childhood hemophagocytic lymphohistiocytosis based on clinical features at diagnosis: A 13-year single-institute retrospective study in Thailand
    Pattranan Kusontammarat, Chane Choed-Amphai, Lalita Sathitsamitphong, Watchareewan Sontichai, Rungrote Natesirinilkul, Pimlak Charoenkwan
    Annals of Hematology.2024;[Epub]     CrossRef
  • 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
Case report
Hemophagocytic lymphohistiocytosis with recurrent Kikuchi-Fujimoto disease
Sang Min Lee, Young Tae Lim, Kyung Mi Jang, Mi Jin Gu, Jong Ho Lee, Jae Min Lee
Yeungnam Univ J Med. 2021;38(3):245-250.   Published online November 11, 2020
DOI: https://doi.org/10.12701/yujm.2020.00654
  • 6,288 View
  • 108 Download
  • 4 Crossref
AbstractAbstract PDF
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome—HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

Citations

Citations to this article as recorded by  
  • Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults: A single‐center analysis of 5 cases
    Qingqing Chen, Jing Zhang, Huijun Huang, Tonglu Qiu, Ze Jin, Yu Shi, Huayuan Zhu, Lei Fan, Jianyong Li, Wenyu Shi, Yi Miao
    Immunity, Inflammation and Disease.2024;[Epub]     CrossRef
  • A young Saudi female with combined hemophagocytic lympho-histiocytosis and Kikuchi’s disease: A case report
    Kamal Al-Zahrani, Batol Gasmelseed, Hesham Waaer Shadi, Rehab Y AL-Ansari
    SAGE Open Medical Case Reports.2023; 11: 2050313X2311543.     CrossRef
  • Cefalea y fiebre: no todo es lo que parece
    María Pilar Iranzo-Alcolea, Carmen Ariño-Palao, Grisell Starita-Fajardo, Andrés González-García, Cecilia Suárez-Carantoña
    Revista Española de Casos Clínicos en Medicina Interna.2023; 8(2): 105.     CrossRef
  • Kikuchi–Fujimoto disease: literature review and report of four cases
    V. G. Potapenko, V. V. Baykov, А. Yu. Markova, N. B. Mikhailova, A. S. Ter‑Grigoryan, Yu. А. Krivolapov
    Oncohematology.2022; 17(4): 48.     CrossRef
Original Article
Significance of serum total bilirubin as a prognostic factor for hemophagocytic lymphohistiocytosis in childhood.
Hea Kyoung Yang, Gui Joung Song, So Eun Jun
Yeungnam Univ J Med. 2014;31(2):75-81.   Published online December 31, 2014
DOI: https://doi.org/10.12701/yujm.2014.31.2.75
  • 1,897 View
  • 10 Download
AbstractAbstract PDF
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease. Despite of proper treatment and improving treatment regimens, HLH patients still show a fatal prognosis. Therefore the evaluation of prognostic factor is important and there are many studies about hyperbilirubinemia as a prognostic factor in HLH. So we studied the prognostic value of hyperbilirubinemia in HLH children. METHODS: A retrospective analysis was performed about 33 patients who were diagnosed with HLH at Pusan National University Hospital and Yangsan Pusan University Hospital between January 2000 to December 2012. We reviewed the clinical characteristics, laboratory findings, and results of treatment to identify hyperbilirubinemia as a prognostic factor in HLH patients. RESULTS: The median age of patients at diagnosis was 32 months. Most of patients presented with fever, pale appearance, abdominal pain and jaundice. Forty-eight point five percentage of patients showed normal serum bilirubiln level (<2.0 mg/dL) and 51.5% showed hyperbilirubinemia (> or =2.0 mg/dL). In normal serum bilirubin group, 1 patient (6.3%) was relapsed and 1 patient (5.9%) was relapsed in hyperbilirubinemia group. In the hyperbilirubinemia group, the mortality was higher than the normal bilirubin group but, there was no statistical significance. CONCLUSION: As a prognostic factor serum bilirubin at diagnosis in HLH patients, there was no significant correlation between hyperbilirubinemia and poor outcome. But, our study has a limitation that the number of patients is too small and almost showed good prognosis.

JYMS : Journal of Yeungnam Medical Science