Macrophage activation syndrome induced by Staphylococcus Epidermidis in a pediatric patient with cystic fibrosis and familial Mediterranean fever

Article Figures & Data

Download figure
Open in new tab
Download powerpoint
Figure 1
- Therapeutic intervention and laboratory parameters. AST: aspartate aminotransferase, Hb: hemoglobin, PLT: platelet count, WBC: white blood cells

Table 1

- Laboratory values of the patient at admission and during follow-up.

Characteristics	Before treatment	Day 1	Day 2	Day 3	Day 10	Day 20	Day 30
WBC (x10⁹/L)	6540	6470	7850	6060	13990	15340	10670
Hb (g/dL)	9.5	10.1	10.6	10	10.4	11.7	10.6
PLT (mm³)	191000	184000	226000	243000	685000	68000	581000
ESR (mm/hr)	55	24	19	24		4
CRP (mg/dL)	8.72	9.2	4.34	1.4	0.3	0.1	0.4
AST (U/L)	243	277	115	206	17	25	22
LDH (U/L)	1339	1552	1049	690	551	241	260
D-dimer (mg/L)	>35.2	>35.2	12.6	2.47	1.38
Ferritin (mg/L)	7169	10172	3056	1279	661	387
Fibrinogen (mg/L)	264	229	274	211	458	495
Triglycerides (mg/dL)	103	99	139	138

WBC: white blood cell count, Hb: hemoglobin, PLT: platelets, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, AST: aspartate transaminase, LDH: lactate dehydrogenase

Table 2

- Patient’s summarized time line.

Relevant past medical history and interventions
An 8-year-old girl with CF (delF508/delF508, pancreatic insufficiency, and chronic P. aeruginosa colonization) and FMF (M694V/M694V)
Dates	Summaries from initial and follow-up visits	Diagnostic testing	Interventions
March 1, 2021	Start to have fever, cough, and increased sputum. Hospitalization and initiation of treatment for acute pulmonary exacerbation of cystic fibrosis.	Infiltration on chest radiography. P. aeruginosa growth in sputum culture.	IV piperacillin-tazobactam and IV amikacin treatment were initiated.
March 13, 2021	New onset of fever, hypoxia, and deterioration in general condition one day after dental intervention.	COVID-19 PCR test negative. S.epidermidis isolated in blood culture.	IV teicoplanin and IV meropenem were initiated.
March 14, 2021	Patient diagnosed with MAS.	Anemia hyperferritinemia. Hypofibrinogenemia. Elevated transaminases.	IV pulse methylprednisolone 30 mg/kg/day for 3 consecutive days was initiated.
March 29, 2021	Clinical improvement was observed. Patient was discharged after 2 weeks.	Laboratory tests returned to reference ranges.	Corticosteroid therapy was gradually tapered over a period of 3 months.

CF: cystic fibrosis, FMF: familial Mediterranean fever, IV: intravenous, COVID-19: coronavirus disease 2019, MAS: macrophage activation syndrome, PCR: polymerase chain reaction, P. aeruginosa: Pseudomonas aeruginosa, S.epidermidis: Staphylococcus epidermidis