Hemophagocytic lymphohistiocytosis: Uncommon systemic inflammatory clinical syndrome

Papa Rao N, Rama Krishna T, Narayana Babu K, Vedaswi Rao V, Bhanu Kiran C, Mayuri S, Shilpa, Ujwal Teja Y

Pdf Page Numbers :- 177-180

 ¹Department of Internal Medicine, Krishna Institute of Medical Sciences, Kondapur, Hyderabad-500084, Telangana, India

²Department of Clinical Pharmacology, Krishna Institute of Medical Sciences, Kondapur, Hyderabad-500084, Telangana, India

³Office of the medical superintendent, Krishna Institute of Medical Sciences, Kondapur, Hyderabad-500084, Telangana, India.

*Corresponding author: Dr. T. Rama Krishna, Clinical Pharmacologist, Krishna Institute of Medical Sciences, Kondapur (Village), Hyderabad-500084, Telangana, India. Mobile: +91-9866468046; Email: ramakrishna.te@kimshospitals.com

Received 03 August 2016; Revised 07 September 2016; Accepted 17 September 2016; Published 29 September 2016

Citation: Paparao N, Ramakrishna T, Narayanababu K, Vedaswirao V, Bhanukiran C, Mayuri S, Shilpa, Teja UY. Hemophagocytic lymphohistiocytosis: Uncommon systemic inflammatory clinical syndrome. J Med Sci Res. 2016; 4(4):177-180. DOI: http://dx.doi.org/10.17727/JMSR.2016/4-039

Copyright: © 2016 Paparao N, et al. Published by KIMS Foundation and Research Center. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Abstract

 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation characterized by clinical signs and symptoms of extreme inflammation. HLH can be challenging to diagnose because the initial symptoms may mimic common infections. People with HLH usually develop symptoms within the first months or years of life which may include fever, enlarged liver or spleen, cytopenia and neurological abnormalities. HLH can only be diagnosed with the proper blood tests. A sample of bone marrow may be obtained to look for hemophagocytosis. Treatment depends on a number of factors, including the severity of symptoms, onset of age, and the underlying cause of the condition. Patients are usually treated with steroids plus chemotherapy and / or an antibody therapy that destroys the T cells. Patients may receive other medications that suppress the immune system. Many patients must also have their immune systems replaced by means of a bone marrow transplant in order to be cured of HLH. We report the brief description of a case along with treatment algorithm.

Keywords: cytopenia, hemophagocytosis, hemophagocytic lymphohistiocytosis