Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion

Table 2

Description of patients according to probable trigger of HPS, diagnostic criteria of HPS, treatment, and evolution.

	Patient 1	Patient 2	Patient 3
Probable trigger of HPS	Abdominal peritonitis	Active tuberculosis or myelodysplastic syndrome	T cell lymphoma
Blood analyses; evolution	Hemoglobin 8,8 g/dL→7 g/dL; Leucocytes >3×10^9/L→1,23 × 10^9/L; Platelet 140×10^9/L→29×10^9/L; Ferritin 1085 μg/L→21091 μg/L; Fasting triglycerides 329 mg/dL; AST 84 U/L; Alkaline phosphatase 192 U/L; C-reactive protein 210 mg/L	Hemoglobin 5,8 g/dL→5 g/dL; Leucocyte 3×10^9/L→0,9 × 10^9/L; Platelet 80×10^9/L→3×10^9/L; Ferritin 19 000 μg/L; Fibrinogen 177 mg/dL; (NR 200–400 mg/dL); 𝛼-Chain of sIL-2—2377 U/mL; (NR 158–623 U/mL); After initial treatment; Hemoglobin 9,5 g/dL; Leucocytes 3,35 × 10^9/L; Platelet 75×10^9/L	Hemoglobin 11,6 g/dL→7,8 g/dL; Leucocyte 2,5 × 10^9/L→0,5 × 10^9/L; Platelet 188×10^9/L→18×10^9/L; Ferritin 11973 μg/L; Fasting triglycerides 345 mg/dL; Total bilirubin 18,12 mg/dL; Direct bilirubin 14,2 mg/dL; ALT 90 U/L; Fibrinogen < 20 mg/dL
Unremitting fever	Yes	Yes	Yes
Spleen enlargement	Yes	No	No
Medullar phagocytosis	Yes	Yes	Yes
Number of HPS criteria	6 in 8	6 in 8	5 in 8
Neurologic symptoms	No	No	No
Involvement of CNS	Unknown	Yes	Unknown
Treatment realized to HPS	Prednisolone 40 mg/m^2	(i) Dexamethasone for 8 weeks with tapering dose from 10 mg/m^2 to 1,25 mg/m^2 dose; (ii) Etoposide 150 mg/m^2 for 8 weeks; (iii) Intrathecal methotrexate (12 mg) in weeks 2, 3, 4, and 5	(i) Methylprednisolone; (ii) Etoposide 150 mg/m^2
Evolution	Dead in 3 days after diagnosis (10 days after admission)	Dead in 4 months after diagnosis	Dead in 4 days after diagnosis (22 days after admission)