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Study Papers | Medicine | India | Volume 13 Issue 5, May 2024 | Rating: 4.5 / 10
Hemophagocytic Leucocytoclastic Histiocytosis: A Rare Case Report
Dr. S. Adhitya Surya
Abstract: Hemophagocytic Leucocytoclastic Histiocytosis (HLH) is a rare and potentially life - threatening hematologic disorder characterized by uncontrolled immune activation and excessive phagocytosis of blood cells. This case report presents a challenging clinical scenario of a 34 - year - old male patient who presented with fever, pancytopenia, hepatosplenomegaly, and skin lesions. Diagnostic workup revealed the presence of hemophagocytosis on bone marrow aspirate and skin biopsy consistent with leucocytoclastic vasculitis. Subsequent investigations demonstrated elevated levels of ferritin, soluble CD25, and triglycerides. Genetic testing revealed a pathogenic mutation in the PRF1 gene. The patient was diagnosed with HLH and promptly initiated on a therapeutic regimen including immunosuppressive therapy and intravenous immunoglobulins. This case underscores the diagnostic challenges and therapeutic complexities of HLH, emphasizing the importance of early recognition and aggressive management in improving patient outcomes.
Keywords: HLH, vasculitis, autoimmune
Edition: Volume 13 Issue 5, May 2024,
Pages: 1559 - 1562