PMC8275851_01
(A) Axillary temperatures as function of age. The highest value per day is shown. The natural course of his disease is also depicted. D, dexamethasone; NICU, neonatal intensive care unit; HLH, hemophagocytic lymphohistiocytosis. He was inpatient during the highlighted periods. (B) Serum levels of C-reactive protein (CRP, mg/l) and ferritin (mug/l) as functions of age. Estimations of the onset (vertical line on Day 100) and duration of his HLH are depicted.
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F1
Thereafter, he developed unremitting fever (Figure 1A) and passed away on Day 145 (Table 1). Results of his serum ferritin and C-reactive protein (CRP) are summarized in Figure 1B.
(A) Color Doppler images on neck ultrasound scan at age of 45 days demonstrates an enlarged left cervical lymph node of increased vascularity in keeping with lymphadenitis. (B) Chest radiograph at age of 145 days shows tube and line in position, and bilateral diffuse alveolar-interstitial opacification with right pleural effusion in keeping with pulmonary edema.
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F2
Briefly, he was admitted on Day 45 with fever and bilateral cervical lymphadenitis (a tender left upper cervical lymph node measuring "2.3 x 1.7 x 1.5 cm" and a right cervical lymph node measuring "1.2 x 0.8 x 0.8 cm"; Figure 2A) and received empiric antibiotics for 10 days. He passed away on Day 145 of multiorgan (hepatic, respiratory, and renal) failure and disseminated intravascular coagulation (DIC), Figure 2B.
Photographs on Day 140 showing (A) his erythematous skin rash, (B) massive hepatomegaly, and (C) BCG site. The BCG vaccination site measured about 5 mm; it was a wet papule with central yellow crust.
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F3
On Day 118, he was admitted with persistent high fever, massive/firm hepatosplenomegaly (spleen 10 cm below the left costal margin and liver down to the pelvis), skin rash, failure to gain weight, and an unhealed BCG injection site (Figure 3).