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Published on: September 5, 2017
Childhood tuberculosis presenting with haemophagocytic syndrome
1Clinical Haematologist, Command Hospital, Udhampur, J&K India.
Insights
Prompt diagnosis and treatment of haemophagocytic syndrome, a severe immune complication of infection, can improve outcomes. This case highlights tuberculosis as a trigger and the effectiveness of combined immunosuppressive and anti-tubercular therapies.
Area of Science:
- Pediatrics
- Immunology
- Infectious Diseases
Background:
- Haemophagocytic syndrome, a life-threatening complication of systemic infection, arises from an exaggerated immune response.
- Early recognition and intervention are critical to reduce the high mortality associated with this condition.
Observation:
- A 2-year-old girl presented with acute enteritis, progressing to prolonged fever, organomegaly, and multi-organ failure.
- Diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) were met, including bone marrow evidence of haemophagocytosis.
- Serological evidence of tuberculosis and a family history of tuberculosis were noted.
Findings:
- The patient's presentation fulfilled the diagnostic criteria for haemophagocytic lymphohistiocytosis.
- Co-existing tubercular infection was identified through serological testing and family history.
- Rapid clinical improvement was observed following initiation of immunosuppressive and anti-tubercular therapies.
Implications:
- This case underscores the association between tuberculosis and haemophagocytic syndrome.
- Prompt diagnosis and combined therapeutic strategies (immunosuppressive and anti-tubercular) can lead to favorable outcomes in pediatric HLH.
- Highlights the importance of considering infectious triggers, such as tuberculosis, in cases of HLH.
Abstract:
Haemophagocytic syndrome is a life threatening complication of systemic infection resulting from an exaggerated immune response to a triggering agent. Prompt recognition and treatment of this disorder can abrogate otherwise high fatality associated with this disorder. A 2 year old girl presented with acute enteritis, developed prolonged fever and organomegaly complicated by multi-organ failure. She fulfilled the diagnostic criteria for haemophagocytic lymphohistiocytosis including bone marrow evidence of haemophagocytosis. In addition she had serological evidence of tubercular infection as well as a positive family history of tuberculosis. She responded rapidly to immunosuppressive therapy and anti-tubercular therapy. Our case illustrates the association of haemophagocytic syndrome with tuberculosis as well as the favourable response obtained with prompt diagnosis and treatment.
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