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Indian Pediatr 2015;52: 78 |
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Hemophagocytic Lymphohistiocytosis Prior to
the Diagnosis of Kawasaki Disease
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Masato Ogawa and *Takayuki
Hoshina
Department of Pediatrics, School of Medicine,
University of Occupational and Environmental Health, Kitakyushu, Japan.
Email:
[email protected]
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We read the recent case report regarding macrophage activation syndrome
secondary to Kawasaki disease [1]. We report a unique case with
hemophagocytosis preceding the diagnosis of Kawasaki disease.
A 9-year-old girl was hospitalized on 7th day of
illness because of prolonged fever and cervical lympha-denopathy. On
admission, leukopenia (total leukocyte count 1.1×10 9/L)
and thrombocytopenia (platelet count 60×109/L)
were present. Serum aspartate amino-transferase (128 U/L), alanine
aminotransferase (99 U/L), lactate dehydrogenase (518 U/L), ferritin
(823 mg/dL), soluble interleukin-2 receptor (1,636 U/mL), interleukin-8
(371 pg/mL) and interferon (IFN)-g
(541 pg/mL) levels were elevated. Triglyceride and C-reactive protein
levels were normal. Coagulation studies revealed increased d-dimer
levels. A bone marrow aspiration revealed appreciable numbers of
hemophagocytosing macrophages. Serology for cytomegalovirus,
Epstein-Barr virus and human parvovirus B19 was negative. On 2nd day of
hospitalization, conjunctival injection, lip erythema and erythematous
papules appeared. There was no hepatosplenomegaly. Echocardiogram showed
dilation of right coronary artery. The patient was diagnosed as having
Kawasaki disease complicated by hemophagocytic lymphohistiocytosis (HLH)
like illness. Clinical symptoms and laboratory findings improved after
the initiations of intravenous immunoglobulin (2 g/kg/dose) and
flurbiprofen (4.5 mg/kg/day). Coronary artery lesion regressed at the
13th day of illness.
HLH generally complicates Kawasaki disease with
prolonged or relapsing course [2]; it developing before the diagnosis of
Kawasaki disease is unusual. The pattern of serum cytokines in the
present patient was similar to those in virus-associated HLH [3].
Increased IFN-g
level suggests the exaggerated systemic inflammatory response to viral
pathogens [3]. Although no pathogen was identified in this case, an
unknown virus might have induced the symptoms.
Acknowledgements: Drs Masahiro Ishii, Rie
Shirayama, Yuko Honda and Tetsuji Sato, Department of Pediatrics, School
of Medicine, University of Occupational and Environmental Health; and
Prof. Koichi Kusuhara, Department of Pediatrics, School of Medicine,
University of Occupational and Environmental Health, Kitakyushu, Japan.
References
1. Mukherjee D, Pal P, Kundu R, Niyogi P. Macrophage
activation syndrome in Kawasaki disease. Indian Pediatr. 2014;51:148-9.
2. Kim HK, Kim HG, Cho SJ, Hong YM, Sohn S, Yoo ES,
et al. Clinical characteristics of hemophagocytic
lymphohistiocytosis related to Kawasaki disease. Pediatr Hematol Oncol.
2011;28:230-6.
3. Ohga S, Matsuzaki A, Nishizaki M, Nagashima T, Kai
T, Suda M, et al. Inflammatory cytokines in virus-associated
hemophagocytic syndrome. Interferon-gamma as a sensitive indicator of
disease activity. Am J Pediatr Hematol Oncol. 1993; 15: 291-8.
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