Adult hemophagocytic lymphohistiocytosis triggered by disseminated tuberculosis and Klebsiella pneumoniae co-infection in an immunocompetent individual-a diagnostic challenge

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.