Fatal Hemophagocytic Lymphohistiocytosis in a Patient with Miliary Tuberculosis: a Case Report

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome induced by cytotoxic T-cells. Mostly, HLH is secondary to infections, malignancies, or autoimmune disorders. HLH triggered by miliary tuberculosis is rare and mortality rates are high. We report a case of a 58-year-old, Caucasian patient admitted to the ICU with respiratory failure. After extensive tests, the diagnosis of HLH was made. Despite aggressive treatment with antibiotics, etoposide, anakinra, and tocilizumab, our patient succumbed to the illness after 18 days in the ICU. Postmortem, a diagnosis of miliary tuberculosis was made, despite negative PCR and culture of mycobacteria during clinical course. Our case demonstrates the challenges of early diagnosis of HLH and the importance of considering miliary tuberculosis as a possible underlying trigger.

Supplementary information: The online version contains supplementary material available at 10.1007/s42399-022-01232-y.

Keywords: Case report; Hemophagocytic lymphohistiocytosis; Intensive care units; Tuberculosis.

Fig. 1

Histopathological features of the bone marrow showing hemophagocytosis

Fig. 2

Overview of histopathological findings during autopsy. a Imaging of the lung showing millet-like grains. b, c Microscopic photograph of granuloma in the lung and liver. d Hemophagocytosis in bone marrow. e, f Positive Ziehl–Neelsen staining in the lung