Secondary Hemophagocytic Lymphohistiocytosis: A Series of Three Cases

Hemophagocytic lymphohistiocytosis (HLH) is a disease of abnormal activation of the immune system, either due to a familial cause or a sporadic cause, in relation to various triggering agents. Secondary HLH is a complication of various diseases, such as infections, malignancies, and autoimmune disorders. In our case series, we present three cases of secondary HLH with varied etiologies. Case 1 involved an 18-year-old male with a history of pulmonary tuberculosis, presenting with fever, hepatosplenomegaly, and elevated inflammatory markers. HLH was treated with steroids alongside antitubercular therapy (ATT). In case 2, a 17-year-old male presented with dengue fever, fever, hepatosplenomegaly, and elevated inflammatory markers. HLH was managed with steroids and etoposide. In case 3, a 29-year-old female with systemic lupus erythematosus (SLE) presented with fever, hepatosplenomegaly, and a positive antinuclear antibody (ANA) test. Steroid therapy was initiated for HLH. The prognosis depends on various factors. The management of such cases necessitates expeditious treatment of the underlying disease in conjunction with amelioration of the cytokine storm with the immunosuppressive agents precipitated by the secondary conditions. Once the underlying cause of the cytokine storm is treated, the lethal progression of the disease may come to a halt.