Tuberculosis associated hemophagocytic lymphohistiocytosis (TB-HLH)-A case series and role of steroid pulse therapy & intravenous immunoglobulin (IVIG) in a critically ill TB-HLH patient.

HLH is a life-threatening hyper-inflammatory condition of uncontrolled, ineffective activation of immune cells leading to hemophagocytosis, and damage to multiple organs. Viral, bacterial, tubercular, fungal, parasitic infections, including malignancy and immune disorders are responsible for secondary HLH. In this case series all four cases presented in life threatening condition. All were diagnosed and treated timely with favourable outcome in three cases. ATT is the mainstay of treatment. Corticosteroids are typically the first-line immunosuppressive agents used in TB-HLH. When a low dose steroid appear inadequate, escalation to a higher dose may result in a favourable clinical response. Intravenous immunoglobulin (IVIG), has shown positive outcome in a critically ill patient of our case series. IVIG has immunomodulatory effects and acts on multiple sites, including phagocytosis of mycobacteria. Awareness of tuberculosis associated HLH (TB-HLH) among physicians is needed in regions with a high burden of tuberculosis. TB-HLH can rapidly progress to life-threatening condition and needs early suspicion. Empirical ATT along with steroid may start when there is strong clinical and radiological suspicion, even if initial microbiological results are negative and complete diagnostic criteria of HLH not fulfilled. Keywords Tuberculosis, Hemophagocytic, lymphohistiocytosis, Corticosteroid, Intravenous immunoglobulin.