International Journal of Advances in Medicine | October 2022 | Vol 9 | Issue 10 Page 1064 International Journal of Advances in Medicine Kondam A et al. Int J Adv Med. 2022 Oct;9(10):1064-1068 http://www.ijmedicine.com pISSN 2349-3925 | eISSN 2349-3933 Case Report Adult hemophagocytic lymphohistiocytosis triggered by disseminated tuberculosis and Klebsiella pneumoniae co-infection in an immunocompetent individual-a diagnostic challenge Alekya Kondam 1 , Mukhyaprana Prabhu 1 *, Shreesha N. 1 , Madhavan Vidarshan 1 , Sindhura Lakshmi 2 INTRODUCTION Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome characterized by excessive activation of macrophage and T-cells, causing excessive cytokines production and subsequent immune-mediated injury of different body organs. 1 It usually occurs secondary to malignancy, infections, or autoimmune disorders. HLH is marked by cytopenias and signs and symptoms of systemic inflammation related to macrophage activation. 2 This diagnosis is gaining importance as its presentation is quite similar to sepsis and sepsis-like conditions and can get overlooked easily. Disseminated tuberculosis occurs by hematogenous spread of Mycobacterium tuberculosis from lung and may involve bone, bone marrow, central nervous system, liver, spleen, skin etc. Patients usually presented with fever, anorexia, respiratory ailments in most of the cases. HLH might rarely complicate the clinical course of disseminated TB. Recent literature review by Padhi et al reported only 63 cases of tuberculosis-associated HLH up to year 2014. 3 Klebsiella pneumoniae is a serious gram-negative bacillus commonly causing hospital acquired pneumonia, urinary tract infections and so sepsis and multi-organ dysfunction. However, Klebsiella pneumoniae was associated to ABSTRACT Here in we report a diagnostically challenging case of adult hemophagocytic lymphohistiocytosis (HLH) triggered by disseminated tuberculosis and Klebsiella pneumoniae co-infection in an immunocompetent Individual. She was a young female presented with complaints of fever, abdominal pain and jaundice. Her evaluation showed cytopenias, hyperbilirubinemia, transaminitis, and hepatosplenomegaly. She progressed to have multi-organ involvement in the form of myocarditis, pleural effusion. Provisional diagnosis of fever with unknown origin and sepsis with multiple- organ dysfunction was made and evaluated for the same. Rapid clinical deterioration with evaluation for sepsis being normal prompted for considering HLH in the differential diagnoses, bone marrow and other criteria have been met resulting in confirmation of the same. Without prior past or family history of HLH, secondary HLH was suspected and substantial evaluation for possible triggers was made, and concomitantly immune suppression was started with corticosteroids. Disseminated tuberculosis was diagnosed and concomitantly Klebsiella pneumoniae was isolated from the bronchioalveolar lavage cultures. As there was no significant immune response culmination, intravenous immunoglobulins were added along with the treatment for possible triggers-tuberculosis and Klebsiella simultaneously. Patient showed significant improvement with this approach. In conclusion management of HLH is different from conventional sepsis and the treatment for each cause of HLH also varies. Furthermore, this case report stresses on the importance for initiating treatment rapidly and tailored approach of management therapy for each case. Keywords: HLH, Klebsiella pneumoniae, Disseminated tuberculosis 1 Department of General Medicine, 2 Department of Pathology, Kasturba Medical College, Manipal, Karnataka, India Received: 18 August 2022 Accepted: 15 September 2022 *Correspondence: Dr. Mukhyaprana Prabhu, E-mail: mm.prabhu@manipal.edu Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: https://dx.doi.org/10.18203/2349-3933.ijam20222407