Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
권호 표지
DOI QR코드

DOI QR Code

Differentiation between incomplete Kawasaki disease and secondary hemophagocytic lym­phohistiocytosis following Kawasaki disease using N­-terminal pro­-brain natriuretic peptide

  • Choi, Jung Eun (Department of Pediatrics, Ewha Womans University School of Medicine) ;
  • Kwak, Yujin (Department of Pediatrics, Ewha Womans University School of Medicine) ;
  • Huh, Jung Won (Department of Laboratory Medicine, Ewha Womans University School of Medicine) ;
  • Yoo, Eun-Sun (Department of Pediatrics, Ewha Womans University School of Medicine) ;
  • Ryu, Kyung-Ha (Department of Pediatrics, Ewha Womans University School of Medicine) ;
  • Sohn, Sejung (Department of Pediatrics, Ewha Womans University School of Medicine) ;
  • Hong, Young Mi (Department of Pediatrics, Ewha Womans University School of Medicine)
  • 투고 : 2017.08.25
  • 심사 : 2018.03.17
  • 발행 : 2018.05.15
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome with many causes, including Kawasaki disease (KD). The purpose of this study was to identify the laboratory tests needed to easily differentiate KD with HLH from incomplete KD alone. Methods: We performed a retrospective study on patients diagnosed with incomplete KD and incomplete KD with HLH (HLH-KD) between January 2012 and March 2015. We compared 8 secondary HLH patients who were first diagnosed with incomplete KD with all 247 incomplete KD diagnosed patients during the study period. The complete blood count, erythrocyte sedimentation rate, platelet count, and serum total protein, albumin, triglyceride, C-reactive protein, N-terminal pro-brain natriuretic peptide (NT-proBNP), and ferritin levels were compared. Clinical characteristics and echocardiography findings were also compared between the 2 groups. Results: The total duration of fever was longer in the HLH-KD group than in the KD group. White blood cell and platelet counts were higher in the KD group. Alanine aminotransferase, ferritin, and coronary artery diameter were increased in the HLH-KD group compared with those in the KD group. The median of NT-proBNP was significantly higher in the HLH-KD group than in the KD group at 889.0 (interquartile range [IQR], 384.5-1792.0) pg/mL vs. 233.0 (IQR, 107.0-544.0) pg/mL. Conclusion: The NT-proBNP level may be helpful in distinguishing incomplete KD from KD with HLH. The NT-proBNP level should be determined in KD patients with prolonged fever, in addition to the white blood cell count, platelet count, and ferritin level, to evaluate secondary HLH.

키워드

참고문헌

  1. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long­term management of kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 2017;135:e927-­99. https://doi.org/10.1161/CIR.0000000000000484
  2. Kang HR, Kwon YH, Yoo ES, Ryu KH, Kim JY, Kim HS, et al. Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease. Blood Res 2013;48:254-­7. https://doi.org/10.5045/br.2013.48.4.254
  3. Mukherjee D, Pal P, Kundu R, Niyogi P. Macrophage activation syn­ drome in Kawasaki disease. Indian Pediatr 2014;51:148-­9.
  4. Yang SL, Xu XJ, Tang YM, Song H, Xu WQ, Zhao FY, et al. Asso­ ciations between inflammatory cytokines and organ damage in pedi­ atric patients with hemophagocytic lymphohistiocytosis. Cytokine 2016;85:14-­7. https://doi.org/10.1016/j.cyto.2016.05.022
  5. Palazzi DL, McClain KL, Kaplan SL. Hemophagocytic syndrome after Kawasaki disease. Pediatr Infect Dis J 2003;22:663-­6.
  6. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 2001;85:421-­6. https://doi.org/10.1136/adc.85.5.421
  7. Muise A, Tallett SE, Silverman ED. Are children with Kawasaki disease and prolonged fever at risk for macrophage activation syndrome? Pediatrics 2003;112(6 Pt 1):e495. https://doi.org/10.1542/peds.112.6.e495
  8. al-­Eid W, al-­Jefri A, Bahabri S, al­-Mayouf S. Hemophagocytosis complicating Kawasaki disease. Pediatr Hematol Oncol 2000;17:323­-9. https://doi.org/10.1080/088800100276316
  9. Kaneko K, Takahashi K, Fujiwara S, Maruyama T, Obinata K. Kawa­ saki disease followed by haemophagocytic syndrome. Eur J Pediatr 1998;157:610­-1. https://doi.org/10.1007/s004310050893
  10. Ohga S, Ooshima A, Fukushige J, Ueda K. Histiocytic haemophago­ cytosis in a patient with Kawasaki disease: changes in the hyper­ cytokinaemic state. Eur J Pediatr 1995;154:539-­41. https://doi.org/10.1007/BF02074830
  11. Cummings C, McCarthy P, van Hoff J, Porter G Jr. Kawasaki disease associated with reactive hemophagocytic lymphohistiocytosis. Pediatr Infect Dis J 2008;27:1116-­8. https://doi.org/10.1097/INF.0b013e31817ecb6d
  12. Titze U, Janka G, Schneider EM, Prall F, Haffner D, Classen CF. Hemophagocytic lymphohistiocytosis and Kawasaki disease: com­ bined manifestation and differential diagnosis. Pediatr Blood Cancer 2009;53:493­-5. https://doi.org/10.1002/pbc.22098
  13. Filipovich AH. Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology Am Soc Hematol Educ Program 2009:127­-31.
  14. Demirkol D, Yildizdas D, Bayrakci B, Karapinar B, Kendirli T, Koroglu TF, et al. Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunc­ tion syndrome/macrophage activation syndrome: what is the treat­ ment? Crit Care 2012;16:R52. https://doi.org/10.1186/cc11256
  15. Sackett K, Cunderlik M, Sahni N, Killeen AA, Olson AP. Extreme Hyperferritinemia: causes and impact on diagnostic reasoning. Am J Clin Pathol 2016;145:646-­50. https://doi.org/10.1093/ajcp/aqw053
  16. Dahdah N, Siles A, Fournier A, Cousineau J, Delvin E, Saint­Cyr C, et al. Natriuretic peptide as an adjunctive diagnostic test in the acute phase of Kawasaki disease. Pediatr Cardiol 2009;30:810-­7. https://doi.org/10.1007/s00246-009-9441-2
  17. Bae HK, Lee DK, Kwon JH, Kim HS, Sohn S, Hong YM. Clinical characteristics and serum N­terminal pro­brain natriuretic peptide as a diagnostic marker of Kawasaki disease in infants younger than 3 months of age. Korean J Pediatr 2014;57:357-­62. https://doi.org/10.3345/kjp.2014.57.8.357
  18. Lin KH, Chang SS, Yu CW, Lin SC, Liu SC, Chao HY, et al. Usefulness of natriuretic peptide for the diagnosis of Kawasaki disease: a syste­ matic review and meta­analysis. BMJ Open 2015;5:e006703. https://doi.org/10.1136/bmjopen-2014-006703
  19. Kwon H, Lee JH, Jung JY, Kwak YH, Kim DK, Jung JH, et al. N­ter­ minal pro­brain natriuretic peptide can be an adjunctive diagnostic marker of hyper­acute phase of Kawasaki disease. Eur J Pediatr 2016; 175:1997­2003. https://doi.org/10.1007/s00431-016-2798-3
  20. Japan Kawasaki Disease Research Committee. Report of subcommittee on standardization of diagnostic criteria and reporting of coronary artery lesions in Kawasaki disease. Tokyo: Ministry of Health and Welfare, 1984.
  21. Freeman HR, Ramanan AV. Review of haemophagocytic lympho­ histiocytosis. Arch Dis Child 2011;96:688­93. https://doi.org/10.1136/adc.2009.176610
  22. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH­2004: Diagnostic and therapeutic guidelines for hemo­ phagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124­-31. https://doi.org/10.1002/pbc.21039
  23. Brisse E, Wouters CH, Matthys P. Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities. Br J Haematol 2016;174:203-­17. https://doi.org/10.1111/bjh.14147
  24. Chen Y, Shang S, Zhang C, Liu T, Yang Z, Tang Y. Hemophagocytic lymphohistiocytosis at initiation of kawasaki disease and their differ­ ential diagnosis. Pediatr Hematol Oncol 2010;27:244-­9. https://doi.org/10.3109/08880011003623642
  25. Inoue S, Mangat C, Rafe'e Y, Sharman M. Forme Fruste of HLH (hae­ mophagocytic lymphohistiocytosis): diagnostic and therapeutic challenges. BMJ Case Rep 2015 Jan 29;2015. pii: bcr2014206190. https://doi.org/10.1136/bcr­2014­206190.
  26. Kleynberg RL, Schiller GJ. Secondary hemophagocytic lympho­ histiocytosis in adults: an update on diagnosis and therapy. Clin Adv Hematol Oncol 2012;10:726-­32.
  27. Sung L, King SM, Carcao M, Trebo M, Weitzman SS. Adverse outcomes in primary hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol 2002;24:550-­4. https://doi.org/10.1097/00043426-200210000-00011
  28. Karapinar B, Yilmaz D, Balkan C, Akin M, Ay Y, Kvakli K. An unusual cause of multiple organ dysfunction syndrome in the pediatric inten­ sive care unit: hemophagocytic lymphohistiocytosis. Pediatr Crit Care Med 2009;10:285-­90. https://doi.org/10.1097/PCC.0b013e318198868b
  29. Beutel G, Wiesner O, Eder M, Hafer C, Schneider AS, Kielstein JT, et al. Virus­associated hemophagocytic syndrome as a major contributor to death in patients with 2009 influenza A (H1N1) infection. Crit Care 2011;15:R80. https://doi.org/10.1186/cc10073
  30. Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lan­ cet Infect Dis 2007;7:814-­22. https://doi.org/10.1016/S1473-3099(07)70290-6

피인용 문헌

  1. Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis vol.36, pp.1, 2019, https://doi.org/10.1080/08880018.2019.1572253
  2. Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome vol.8, pp.4, 2021, https://doi.org/10.3390/children8040269
  3. Kawasaki Disease: A Systematic Review and Meta‐Analysis of Benefits and Harms of Common Treatments vol.3, pp.10, 2021, https://doi.org/10.1002/acr2.11308