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RGUHS Nat. J. Pub. Heal. Sci Vol: 14  Issue: 2 eISSN:  pISSN

Case Report

Haemophagocytic Lymphohistiocytosis

Muralidhar L.Kulkarni 1, Sidramayya SS 2, Vivekanand VV2 , Anish Desai 2, Hemalatha2

1Professor and Head, 2Resident, Department of Pediatrics, JJM Medical College, Davangere

Year: 2013, Volume: 3, Issue: 1, Page no. 59-62,
Views: 620, Downloads: 2
Licensing Information:
CC BY NC 4.0 ICON
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0.
Abstract

Haemophagocytic lymphohistiocytosis is a rare macrophage related hyperinflammatory disorder characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections, collagen vascular disease and malignancies. The pathological hallmark of this disorder is aggressive proliferation of macrophages and histiocytes. The symptoms are due to high levels of cytokines secreted by immune effector cells which display functional defects. Recognizing the morphologic changes in the liver and the immunophenotypic feature of the infiltrate are critical for a rapid diagnosis and prompt initiation of treatment.We report a case of haemophagocytic lymphohistiocytosis as it is one of the emerging infectious diseases and review current literature.

<p>Haemophagocytic lymphohistiocytosis is a rare macrophage related hyperinflammatory disorder characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections, collagen vascular disease and malignancies. The pathological hallmark of this disorder is aggressive proliferation of macrophages and histiocytes. The symptoms are due to high levels of cytokines secreted by immune effector cells which display functional defects. Recognizing the morphologic changes in the liver and the immunophenotypic feature of the infiltrate are critical for a rapid diagnosis and prompt initiation of treatment.We report a case of haemophagocytic lymphohistiocytosis as it is one of the emerging infectious diseases and review current literature.</p>
Keywords
haemophagocytosis, Histiocytosis,
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