Abstract
Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation. It commonly affects infants from birth to 18 months of age, but cases in older children and adults have also been reported. Key players in HLH are activated lymphocytes and histiocytes, they infiltrate all organs and secrete large amounts of cytokines. Cardinal symptoms are prolonged fever, hepatosplenomegaly, cytopenias and hemophagocytosis [1]. The aim of this case presentation is to focus on the unique presentations of HLH syndrome and to be aware of unusual presentations, so that we could diagnose early and treat it appropriately to ensure long term survival.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation
A series of adult patients with Haemophagocytic lymphohistiocytosis (HLH) and a variety of autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still’s disease, polyarteritis nodosa, mixed connective tissue disease, pulmonary sarcoidosis, systemic sclerosis, and Sjogren’s syndrome have been reported. This is the first case reported of HLH syndrome presenting as Polymyositis
Soluble CD25 > 2400 U/ml There are published observational data suggest that the TNF inhibitor etanercept is potentially useful for obtaining remission in children not responding to steroids and cyclosporin A [4]
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation. Case presentation 19 yr old young boy presented with high fever, rigors & lethargy 5 weeks after been to Spain. Investigations showed neutropenia, lymphopenia & abnormal LFT’s (WCC 1.2, Neut 0.7, ALT 815, ESR 13, CRP
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