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Haemophagocytic Lymphohistiocytosis: A Review Article

Dr. Sreekantham Vinay Kumar, Dr. Arti Muley

Abstract: Haemophagocytic Lymphohistiocytosis: Review article. Abstract: Background Haemophagocytic lymphohistiocytosis (HLH) is a hyper?inflammatory syndrome characterized by the aggressive non-malignant proliferation of activated macrophages and histiocytes, which phagocytose other cells, namely red blood cells, white blood cells and platelets leading to the clinical symptoms of fever, hepato-splenomegaly, bone marrow, skin, and central nervous system (CNS) infiltration. The presumed underlying pathogenesis is a highly?stimulated but ineffective immune response to antigens, mediated mainly by activated T?cells and associated macrophage activation that eventually heightens to a life?threatening cytokine storm. Interpretation: In this case series, we present 2 cases of HLH their different presentations, clinical course and outcome. Conclusion: HLH is not an uncommon entity and it has multifaceted clinical presentation with nonspecific signs and symptoms that are often found in other clinical conditions. Therefore, there is need to know the importance of its Incidence in Indian scenario, specific management guidelines and prognosis to prevent high mortality and adverse outcomes.

Keywords: Fever Of Unknown Origin, Haemophagocytic Lymphohistiocytosis, Hypertriglyceridemia, Hepatosplenomegaly,Pancytopenia, Serum Ferritin.

Country: India, Subject Area: Hematology Science

Pages: 661 - 665

Edition: Volume 8 Issue 6, June 2019

How to Cite this Article?

Dr. Sreekantham Vinay Kumar, Dr. Arti Muley, "Haemophagocytic Lymphohistiocytosis: A Review Article", International Journal of Science and Research (IJSR), https://www.ijsr.net/archive/v8i6/show_abstract.php?id=ART20198390, Volume 8 Issue 6, June 2019, 661 - 665

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