Infected-associated hemophagocytic syndrome

Area of Science:

• Pediatrics
• Infectious Diseases
• Hematology

Background:

• An epidemic of a novel infection characterized by circulating hemophagocytes (HP) and activated monocytes (AM) occurred in Bombay.
• The condition presented distinct features overlapping with, yet separate from, virus-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis.

Purpose of the Study:

• To describe the clinical and laboratory features of this distinct hemophagocytic syndrome in children.
• To categorize the affected children into distinct groups based on age and clinical presentation.

Main Methods:

• Observational study of children presenting with the novel infection in Bombay.
• Clinical assessment including age, symptoms, neurological status, and bleeding.
• Laboratory investigations including blood smears, coagulation profiles, and biochemical tests.
• Subgroup analysis based on age (neonates, infants, children) and surgical cases with small bowel malformations.

Main Results:

• Affected children (2 days to 2 years) exhibited fever, altered sensorium, neurological symptoms, dyspnea, diarrhea, and significant bleeding.
• Key laboratory findings included neutrophilia, presence of AM and HP in blood smears, coagulopathy, normal CSF and liver transaminases, hypertriglyceridemia, and hypoalbuminemia.
• Surgical cases were associated with small bowel malformations. Four distinct clinical groups were identified: neonates, infants, children, and surgical.

Conclusions:

• A distinct epidemic infection involving hemophagocytes and activated monocytes occurred in Bombay, primarily affecting young children.
• The condition presents with a unique constellation of symptoms and laboratory findings, necessitating its classification separate from established hemophagocytic syndromes.
• Clinical presentation and outcomes vary significantly across different age groups and in surgical cases with gastrointestinal anomalies.