Clinical presentation of hemophagocytic lymphohistiocytosis in adults is less typical than in children

• Published in: Clinics (São Paulo, Brazil) Vol. 71; no. 4; pp. 205 - 209
• Main Authors: Zhang, Zuojuan, Wang, Juandong, Ji, Buqiang, von Bahr Greenwood, Tatiana, Zhang, Yuan, Wang, Yongjing, Kong, Dexiao, Li, Ai, Jiang, Yang, Guo, Yanan, Liu, Xiaoli, Wang, Yingxue, Dou, Aixia, Li, Nailin, Henter, Jan-Inge, Sun, Guizhen, Zheng, Chengyun
• Format: Journal Article
• Language: English
• Published: Brazil Elsevier España, S.L.U 01.04.2016; Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo; Faculdade de Medicina / USP; Elsevier España
• Subjects: Adolescent; Adult; Age Factors; Aged; Child, Preschool; China - epidemiology; Clinical Characteristics; Clinical Science; Drug Therapy, Combination; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - diagnosis; Female; Hemophagocytic Lymphohistiocytosis; Hepatomegaly - epidemiology; Humans; Infant; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphohistiocytosis, Hemophagocytic - etiology; Male; Medicin och hälsovetenskap; MEDICINE, GENERAL & INTERNAL; Middle Aged; Pediatric; Splenomegaly - epidemiology; Young Adult; Adult; Hemophagocytic Lymphohistiocytosis; Clinical Characteristics; Pediatric
• ISSN: 1807-5932; 1980-5322; 1980-5322
• DOI: 10.6061/clinics/2016(04)05

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. Data from 50 hemophagocytic lymphohistiocytosis patients, including 34 adults and 16 children who fulfilled the 2004 hemophagocytic lymphohistiocytosis diagnostic criteria, were collected and analyzed. 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). 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.