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A previously healthy 3½12 month old infant was admitted to the hospital with dyspnoea, malaise, and irritability in August 2001. One week before he had had an upper respiratory infection treated with amoxycillin and inhaled steroids; the day before admission he presented high grade (up to 40°C) temperature.
On admission he was febrile (39°C), pale, restless; his respiratory rate was 50/min and pulse 120 beats/min. Clinical evaluation showed oral cyanosis, redness of the pharynx, and bilateral suppurative conjunctivitis. A chest radiograph showed pulmonary infiltrates in the right lung.
Electrocardiography (ECG) and echo colour Doppler excluded cardiac abnormalities. An electroencephalogram showed diffuse slow waves. A lumbar puncture was performed to exclude a meningeal infection; the cerebrospinal fluid was clear with normal glucose and protein levels and a mild increase in mononuclear cells.
Blood tests showed: erythrocyte sedimentation rate (ESR) (Westergren) 120 mm/1st h, C reactive protein (CRP) 221 mg/l, white blood cells 16.8×109/l (neutrophils 75.2%), haemoglobin 94 g/l, and platelet count 504×109/l. Serum urea, creatinine, electrolytes, transaminases, γ-glutamyltransaminase, IgA, IgM, IgG, lactate, pyruvate, lactate dehydrogenase levels, and blood and urine cultures were normal. Serological tests for antibodies to measles, mumps, chickenpox, herpes viruses, adenovirus, HIV, hepatitis C and B, cytomegalovirus, Mycoplasma pneumoniae, Leishmania were all negative. Vanillylmandelic acid in the urine was in the normal range.
Despite wide spectrum antibiotic treatment (netilmicin, ceftazidime) his general condition deteriorated and he was less alert and extremely pale. He lost weight and developed liver, spleen, and cervical lymph node enlargement.
On day 5 from admission, a diffuse maculopapular rash affecting all the body and the scalp was evident. Over the following days the child had diarrhoea and developed generalised oedema. Fever persisted (up to 39.5°C).
Abdominal ultrasound confirmed hepatosplenomegaly and excluded the presence of other masses. Cardiac evaluation, with ECG and echocardiography performed on days 7 and 10 was normal.
Blood tests were repeated and showed an increased ESR (132 mm/1st h) and CRP (231 mg/ll), and decreased haemoglobin (60 g/l), fibrinogen 4.5 g/l, and platelet count 116×109/l. After blood transfusion, the child underwent abdominal and chest computed tomographic scan that showed the presence of multiple lymph nodes in the retroperitoneal, cervical, and axillar areas.
Lung infiltrates were still present. As a peripheral blood smear showed the presence of myelocytes and promyelocytes a bone aspiration was performed, and several histiocytes were found. Because a lymphoproliferative disorder was suspected a bone marrow biopsy was planned. During anaesthesia the boy underwent cardiac arrest and was transferred to the intensive care unit.
ECG showed inverted T waves and echo colour Doppler two giant aneurysms: the left coronary diameter was 13 mm and the right coronary diameter 11 mm (fig 1); on the posterior left ventricular wall a small area of myocardial necrosis was detected.
The child was then referred to our unit. Kawasaki disease (KD) was then suspected and the appropriate treatment with intravenous immunoglobulin (IVIg) (2 g/kg) was immediately given. Anticoagulation with low weight heparin was also started, owing the appearance of giant aneurisms and myocardial infarction. The fever promptly dropped during IVIg infusion, so high dose aspirin was not started. The patient improved dramatically; he became alert and active.
Blood tests showed a high platelet count 1.2×1012/l. Three weeks after admission the patient’s two digits of his right hand peeled. Blood tests progressively improved and completely normalised over four weeks. In October the child was discharged in general good condition while continuing to receive treatment with oral anticoagulant. At the last control, May 2002, a cardiological evaluation showed a slight reduction of the coronary aneurysms (left coronary diameter 9 mm and right 10 mm), and anticoagulation was continued.
KD is one of the most common systemic vasculitides in childhood. Owing to a lack of diagnostic tests, the diagnosis is based on clinical criteria after the exclusion of other febrile diseases in young children.1
The presence of histiocytes, probably a reactive event, led us to consider the diagnosis of histiocytosis. The differential diagnosis includes sepsis, scarlet fever, toxic shock syndrome, viral infections, in particular due to enterovirus, adenovirus, measles, parvovirus, cytomegalovirus, Epstein-Barr virus, and Mycoplasma pneumoniae2. Lymphoproliferative disorders are usually not included in the differential diagnosis of KD.
In fact, recently an increasing number of children with atypical onset of KD have been reported,3–5 but to our knowledge a patient with clinical signs mimicking a lymphoproliferative disorder has not been described so far.
This case emphasises that KD must be suspected in any child with high, persistent fever, even in absence of all diagnostic criteria; IVIg can be recommended before the typical manifestations are all present.6 Delayed treatment might be responsible for severe coronary complications, and even sudden death, especially in infants.7
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