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IP Journal of Paediatrics and Nursing Science


A case report on HLH in a rural area- A missed diagnosis, challenges and recommendations


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Article Type : Case Report

Author Details: Likhita Shaik*,Sagar Mhetre,Sunil Vaidya

Volume : 2

Issue : 3

Online ISSN : XXXX

Print ISSN : XXXX

Article First Page : 81

Article End Page : 84


Abstract

“Hemophagocytic lymphohistiocytosis (HLH) is a histiocytic disorder characterized by a highly stimulated, but ineffective, immune response to antigens, which results in a fatal cytokine storm with an inflammatory reaction”. It is often underdiagnosed due to its vague presentation and similarities it shares with a spectrum of other diseases. To add to the obstacles, ruling out each causative mechanisms include underlying genetic defects as well as “trigger” factors, such as infections, malignancies, and autoimmune disorders consumes time and hence delays the diagnosis and treatment. Inspite of various advances in genetic testing and analysis diagnosis becomes a challenging event when it occurs in rural areas where very little is known about it. Here we present a case of a 2 month old female presenting with fever, decreased appetite and excessive irritability. The various parts of the Case report highlights the aspects of every barrier that came across to reach the diagnosis. Moreover, it also adds on how the perplexity of the social concerns limit the ability to obtain essential health care and thus paving way for some amendments that can be made to improve the healthcare status in rural areas.

Keywords: FHLH, Health care, HLH, Hemophagocytic lymphohistiocytosis, Rural areas, Recommendations, Reforms, SHLH.