title: Clinical presentation of hemophagocytic lymphohistiocytosis in adults is less typical than in children.
abstract: OBJECTIVE
  Hemophagocytic lymphohistiocytosis in adults is largely underdiagnosed. To improve the rate and accuracy of diagnosis in adults, the clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis were analyzed in and compared between adults and children in a Chinese cohort.
  
  
  METHODS
  Data from 50 hemophagocytic lymphohistiocytosis patients, including 34 adults and 16 children who fulfilled the 2004 hemophagocytic lymphohistiocytosis diagnostic criteria, were collected and analyzed.
  
  
  RESULTS
  1. Etiological factors: The proportion of Epstein-Barr virus infection was lower in adults compared with children, whereas fungal infection and natural killer/T cell lymphoma were more frequent in adults (P<0.05). 2. Clinical manifestations and laboratory findings: Over 90% of adults and pediatric patients presented with fever, thrombocytopenia and high serum ferritin levels. However, in adults, the proportions of hepatomegaly, splenomegaly and jaundice were much lower (P<0.01) than in children, and serous cavity effusion was more frequent in adult patients (P<0.05). More children had hemoglobin <90 g/L, total bilirubin >19 mmol/L and lactate dehydrogenase >500 U/L compared with adults (P<0.05). 3. The time interval from the onset of symptoms to clinical diagnosis was significantly shorter in pediatric patients than in adults (P<0.05).
  
  
  CONCLUSIONS
  Certain clinical features were different between the two groups. The less characteristic clinical presentation of hemophagocytic lymphohistiocytosis in adults may make the disease more difficult to diagnose. Our findings suggest that hemophagocytic lymphohistiocytosis should be considered when an adult patient presents with the above-mentioned symptoms.
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Zuojuan
authors.initials: Z
authors.lastname: Zhang
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Juandong
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Hematology, Linyi People's Hospital, Linyi, China.
authors.forename: Buqiang
authors.initials: B
authors.lastname: Ji
authors.affiliation: Center of Evidence-based Medicine, Shandong University, Jinan, China.
authors.forename: Tatiana von
authors.initials: Tv
authors.lastname: Bahr Greenwood
authors.affiliation: Center of Evidence-based Medicine, Shandong University, Jinan, China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Yongjing
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Dexiao
authors.initials: D
authors.lastname: Kong
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Ai
authors.initials: A
authors.lastname: Li
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Yang
authors.initials: Y
authors.lastname: Jiang
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Yanan
authors.initials: Y
authors.lastname: Guo
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Xiaoli
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Yingxue
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Aixia
authors.initials: A
authors.lastname: Dou
authors.affiliation: Clinical Pharmacology Unit, Department of Medicine-Solna, Karolinska Institutet at Karolinska University Hospital (Solna), Stockholm, Sweden.
authors.forename: Nailin
authors.initials: N
authors.lastname: Li
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Jan-Inge
authors.initials: JI
authors.lastname: Henter
authors.affiliation: Department of Hematology, Linyi People's Hospital, Linyi, China.
authors.forename: Guizhen
authors.initials: G
authors.lastname: Sun
authors.affiliation: Department of Hematology, The Second Hospital of Shandong University, Jinan, China.
authors.forename: Chengyun
authors.initials: C
authors.lastname: Zheng
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D004359:Drug Therapy, Combination; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis; D005260:Female; D006529:Hepatomegaly / Q000453:epidemiology; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008297:Male; D008875:Middle Aged; D013163:Splenomegaly / Q000453:epidemiology; D055815:Young Adult