Korean J Pediatr.  2018 May;61(5):167-173. 10.3345/kjp.2018.61.5.167.

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

• Choi JE
• Kwak Y
• Huh JW
• Yoo ES
• Ryu KH
• Sohn S
• Hong YM

• KMID: 2412144
• DOI: http://doi.org/10.3345/kjp.2018.61.5.167

Abstract

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.

Keyword

Mucocutaneous lymph node syndrome; Hemophagocytic lymphohistiocytosis; Brain natriuretic peptide; Ferritin

MeSH Terms

Alanine Transaminase
Blood Cell Count
Blood Sedimentation
C-Reactive Protein
Coronary Vessels
Echocardiography
Ferritins
Fever
Humans
Leukocyte Count
Leukocytes
Lymphohistiocytosis, Hemophagocytic*
Mucocutaneous Lymph Node Syndrome*
Natriuretic Peptide, Brain
Platelet Count
Retrospective Studies
Triglycerides
Alanine Transaminase
C-Reactive Protein
Ferritins
Natriuretic Peptide, Brain