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Reactive hemophagocytic syndrome presenting as a component of multiple organ dysfunction syndrome
F Gauvin1, B Toledano, J Champagne
1Department of Pediatrics, Sainte Justine Hospital, Université de Montréal, Canada.
Objective:
To report two cases of severe reactive hemophagocytic syndrome (RHS), to discuss their impact, and to present evidence that RHS may be a constitutive part of multiple organ dysfunction syndrome (MODS).
Design:
Case-report.
Setting:
Pediatric intensive care unit (PICU).
Patients:
Two patients with RHS and MODS.
Interventions:
None.
Measurements And Main Results:
Case #1: A 3 yr-old boy with Mucha-Haberman syndrome (pityriasis lichenoides) was admitted to the PICU for septic shock, acute respiratory distress syndrome, capillary leak, acute renal failure, liver dysfunction, and RHS (pancytopenia and hemophagocytosis on bone marrow aspirate). The pancytopenia was severe (white blood cell count, 0.9 x 10(9)/L; hemoglobin, 59 g/L; platelets, 36 x 10(9)/L), required many transfusions, and resolved 2 months later. The patient needed mechanical ventilation for 6 wks. Length of stay in PICU was 2 months. Case #2: A previously healthy 4 yr-old girl was admitted to the PICU for respiratory failure. She developed acute respiratory distress syndrome, cardiomyopathy with complete atrioventricular block, shock, capillary leak, liver dysfunction, and RHS (pancytopenia and hemophagocytosis on bone marrow aspirate). The pancytopenia was severe (white blood cell count, 1.92 x 10(9)/L; hemoglobin, 65 g/L; platelets, 58 x 10(9)/L) and necessitated transfusional support. Serology for respiratory syncytial virus was positive. RHS duration was 20 days; the patient recovered completely. Length of mechanical ventilation was 16 days and length of stay in PICU was 3 wks.
Conclusions:
These cases show that RHS may be a significant cause of pancytopenia in the PICU. It needs to be recognized as a clinical entity because it can be reversible and nonneoplastic. RHS and MODS share some pathophysiologic elements and could be related to each other.
Insights
Reactive hemophagocytic syndrome (RHS) can cause severe pancytopenia in pediatric intensive care units. Recognizing RHS is crucial as it is a reversible, nonneoplastic condition potentially linked to multiple organ dysfunction syndrome (MODS).
Area of Science:
- Pediatric Critical Care Medicine
- Hematology
- Immunology
Background:
- Reactive hemophagocytic syndrome (RHS) is a life-threatening condition characterized by excessive immune activation.
- Multiple organ dysfunction syndrome (MODS) involves the failure of multiple organ systems.
- The relationship between RHS and MODS, particularly in pediatric intensive care settings, requires further elucidation.
Observation:
- Two pediatric cases of severe RHS are presented, both occurring in the context of critical illness.
- Case 1 involved a 3-year-old boy with Mucha-Haberman syndrome presenting with septic shock and MODS.
- Case 2 involved a previously healthy 4-year-old girl with respiratory failure and MODS.
Findings:
- Both patients exhibited severe pancytopenia and hemophagocytosis, indicative of RHS.
- RHS was a significant contributor to the hematologic abnormalities observed.
- One patient experienced a prolonged recovery, while the other recovered completely.
Implications:
- These cases highlight RHS as a critical, potentially reversible cause of pancytopenia in pediatric intensive care units.
- Recognition of RHS is vital for timely and appropriate management.
- The findings suggest a potential pathophysiological link between RHS and MODS, warranting further investigation.