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Hemophagocytic syndrome.

Manjusha Shripad Dhawale1, Anil R Joshi1, Milind Anil Bhatkule1

  • 1Department of Pathology, Government Medical College and Hospital, C/O L.C. Deo 39, SBH Colony, Near Peer Bazaar, Osmanpura, Aurangabad, 431003 Maharashtra India.

Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion
|October 22, 2014
PubMed
Summary

Hemophagocytic syndrome is a severe hyperinflammatory condition often linked to immune deficiencies. This report details a case in an 18-year-old male, offering a literature review.

Keywords:
Hemophagocytic syndromeImmunodeficiency

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Area of Science:

  • Immunology
  • Hematology
  • Critical Care Medicine

Background:

  • Hemophagocytic syndrome (HFS) is a rare, life-threatening hyperinflammatory state.
  • It arises from dysregulated immune responses, often secondary to primary immune deficiencies or acquired triggers.
  • Uncontrolled inflammation and cytokine storm characterize HFS, leading to multi-organ dysfunction.

Observation:

  • A case of HFS is presented in an 18-year-old male patient.
  • The clinical presentation and diagnostic workup are detailed.
  • This case highlights the potential for HFS in young adults.

Findings:

  • The patient experienced severe hyperinflammation consistent with HFS.
  • Diagnostic criteria for HFS were met, necessitating urgent management.
  • The underlying immune deficiency or trigger was investigated.

Implications:

  • Early recognition and prompt treatment of HFS are crucial for patient survival.
  • Understanding the diverse etiologies of HFS is vital for effective management.
  • This case contributes to the literature on HFS, particularly in the adolescent population.