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Flow Cytometric Measurement Of ROS Production In Macrophages In Response To FcγR Cross-linking

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Published on: March 7, 2019

Reactive hemophagocytic syndrome.

L Rajam¹, Vinitha Prasad, B L Yatheesha

¹Department of Pediatrics, Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, India.

Indian Journal of Pediatrics

|September 24, 2008

Summary

This summary is machine-generated.

Reactive Hemophagocytic syndrome (RHS) can manifest in childhood rheumatic diseases like Juvenile Idiopathic Arthritis and Systemic Lupus Erythematosus. Prompt treatment with pulse methylprednisolone therapy led to recovery in both reported cases.

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Area of Science:

Pediatric Rheumatology
Hematology
Immunology

Background:

Reactive Hemophagocytic syndrome (RHS) is a severe, life-threatening hyperinflammatory condition.
RHS is increasingly recognized as a potential complication in various pediatric rheumatic diseases.
Early diagnosis and prompt management are crucial for improving outcomes in pediatric RHS.

Observation:

Two pediatric cases of RHS associated with rheumatic diseases are presented.
Case 1: Adolescent with systemic onset Juvenile Idiopathic Arthritis (JIA) presented with sepsis-like symptoms, shock, liver dysfunction, and coagulopathy.
Case 2: Patient presented with cardiac tamponade and was diagnosed with Systemic Lupus Erythematosus (SLE), with bone marrow biopsy showing hemophagocytosis.

Findings:

Both patients exhibited distinct clinical presentations of RHS.
Hemophagocytosis was a key finding in the bone marrow aspirate of the SLE patient.
Both cases demonstrated significant clinical improvement following treatment with pulse methylprednisolone therapy.

Implications:

This highlights the importance of considering RHS in children with rheumatic diseases presenting with severe systemic symptoms.
Pulse methylprednisolone therapy appears effective in managing RHS in this pediatric population.
Further research into the pathogenesis and optimal management strategies for RHS in pediatric rheumatic diseases is warranted.