Kawasaki Disease Associated With Reactive Hemophagocytic Lymphohistiocytosis

Cummings, Christy L.; McCarthy, Paul L.; Hoff, Jack van; Porter, George A.

doi:10.1097/inf.0b013e31817ecb6d

Cited by 31 publications

(17 citation statements)

References 14 publications

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“…A review of the literature failed to find any case of KD with MAS with a fatal outcome, but numerous children required more intensive treatment than IVIG and steroids alone. [3][4][5][6][7][8] As previously reported, secondary cases of MAS with a rheumatic etiology generally have favorable outcome compared with primary and secondary cases with nonrheumatic etiology, therefore good outcomes in this cohort are not unexpected. 14 The lack of fatal cases in this series or in others does not diminish the seriousness of this disease and those patients should be closely monitored and promptly treated.…”

Section: Discussionsupporting

confidence: 62%

“…1 Rare cases of macrophage activation syndrome (MAS) associated with KD have previously been reported. [2][3][4][5][6][7][8] MAS, also referred to as hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially lifethreatening disease that usually occurs in children and adults with autoimmune diseases, malignancies, or some infections, most notably Epstein-Barr virus. 9 MAS is underrecognized in children.…”

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confidence: 99%

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Macrophage Activation Syndrome in the Acute Phase of Kawasaki Disease

Latino

Manlhiot

Yeung

et al. 2010

Journal of Pediatric Hematology/Oncology

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Rare cases of macrophage activation syndrome (MAS) occurring during the acute phase of Kawasaki disease (KD) have been reported. We sought to characterize, review treatment, and outcomes of KD patients with clinical features of MAS. Medical histories of patients treated for KD and MAS between January 2001 and March 2008 were reviewed. Of 638 KD patients seen, 12 (1.9%) had additional clinical findings usually associated with MAS; 7 of them were males older than 5 years (6.1%; odds ratio: 6.8, P=0.002). Clinically, 9 patients had at least 4 of 5 KD clinical signs, and all patients had prolonged fever beyond initial intravenous immunoglobulin treatment. Hepatosplenomegaly, cytopenia in two or more cell lines, hypertriglyceridemia and/or hypofibrinogenemia, and increased D-dimers were seen in 11 patients. Hyperferritinemia and elevated hepatic enzymes were seen in all patients. Four patients had biopsy-proven evidence of hemophagocytosis. All but 2 patients met at least 5 of 8 criteria necessary for MAS diagnosis. Treatment beyond the standard KD protocol (aspirin + intravenous immunoglobulin) was necessary in all but 1 patient. All patients eventually recovered with no long-term sequelae. A high index of suspicion for clinical features associated with MAS is warranted for KD patients to provide appropriate and timely treatment.

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Section: Discussionsupporting

confidence: 62%

mentioning

confidence: 99%

Macrophage Activation Syndrome in the Acute Phase of Kawasaki Disease

Latino

Manlhiot

Yeung

et al. 2010

Journal of Pediatric Hematology/Oncology

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“…A considerable volume of research has shown that the pathological process of KD is characterized by immune activation and immune vasculitis of the vascular endothelial cells (1). KD mostly affects the small and mediumsized arteries, especially the coronary artery (2), resulting in coronary artery lesion (CAL), such as dilation, aneurysm formation, or advanced stenosis, and it is the leading cause of death in children with KD (3).…”

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confidence: 99%

Plasma MASP-1 concentration and its relationship to recovery from coronary artery lesion in children with Kawasaki disease

Song

Zou

et al. 2015

Pediatr Res

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Background: This study investigated prognostic factors for early recovery of coronary artery lesion (CAL) in children with Kawasaki disease (KD). Methods: Patients hospitalized for KD were enrolled less than 2 wk from the onset of illness and divided into two groups: KD with CAL and KD without CAL. The CAL group was further divided into two subgroups according to the degree of CAL: mild (n = 31) and moderate/severe (n = 6) and further divided into two subgroups according to the age: younger than 1 y (n = 9) and older than 1 y (n = 28). Lectin pathway-related factors MASP-1, CD59, and C5b-9 were measured, along with C-reactive protein, white blood cell counts, erythrocyte sedimentation rate, and platelet count. Patients were followed up for 3 mo. Correlation between the measured factors and the length of time of recovery from CAL was analyzed. results: Plasma concentrations of MASP-1 in the CAL group were significantly lower than those without CAL. MASP-1 and gender positively correlated with the recovery time of CAL. There was no difference in MASP-1 between mild and moderate/severe CAL. At 3-mo follow-up, there was a positive correlation between plasma MASP-1 concentration and recovery time of the patients with CAL older than 1 y. conclusion: Plasma MASP-1 concentration at the early stage of KD is predictive of length of time of recovery from CAL. k awasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute nonspecific systemic vascular inflammatory syndrome occurring mainly in children under 5 y of age. A considerable volume of research has shown that the pathological process of KD is characterized by immune activation and immune vasculitis of the vascular endothelial cells (1). KD mostly affects the small and mediumsized arteries, especially the coronary artery (2), resulting in coronary artery lesion (CAL), such as dilation, aneurysm formation, or advanced stenosis, and it is the leading cause of death in children with KD (3). It has been reported that 15-25% of untreated KD patients may develop CAL (4). Although treatment with intravenous immunoglobulin within 10 d of onset of fever reduces the incidence of CAL, 5-8% of patients still develop it. The degree of CAL varies considerably between individuals, ranging from mild to moderate ectasia of the coronary artery to giant aneurysm formation. The recovery time of CAL also differs between patients. There is a very little information about the length of time for which different degrees of CAL persists. Little is known on the relationship between the complement system and the recovery time of CAL resulting from KD. Our previous study indicated that the lectin pathway of the complement system is activated in KD. CAL is associated with endothelial cell dysfunction induced by T-cell activation (5). Endothelial cells are a source of complement-related factors and complement regulatory factors (6), and MASP-1 activates endothelial cells directly, which then activates the complement lectin pathway (7). Endothelial cell dysfunction (8) and com...

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“…High serum ferritin levels are a feature of HLH. HLH has been described in association with KD in several reports (16)(17)(18)(19)(20)(21). Similarities between the two conditions, and reports of the presence of features of both HLH and KD in some patients may also suggest that the initial inflammatory response manifesting as KD might progress to HLH if untreated.…”

Section: Discussionmentioning

confidence: 76%