Spotted Fever: An Undercover Cause of Hemophagocytic Lymphohistiocytosis in the Immediate Postpartum

Hemophagocytic lymphohistiocytosis (HLH) is characterized by a dysregulated activation of the immune system that causes fever, cytopenias, organomegalies, and hemophagocytosis. There are infectious, neoplastic, rheumatologic, and miscellaneous causes. Rickettsioses are a neglected cause of HLH. We report a confirmed case of an immunocompetent woman in Mexico with postpartum HLH secondary to spotted fever. We did a review of the literature for search of similar cases. The association between these two diseases was found in postmortem studies, unrelated to postpartum. This diagnosis should be considered in all patients with HLH without an evident cause in areas of epidemiological risk.

Automated summary

Hemophagocytic lymphohistiocytosis (HLH) is an immune system disorder characterized by abnormal activation that can be caused by infections, tumors, rheumatologic conditions, and other factors. Rickettsioses, specifically spotted fever, have been found to be a neglected cause of HLH. This report presents a confirmed case of postpartum HLH in an immunocompetent woman in Mexico and highlights the importance of considering this diagnosis in HLH patients without an evident cause in areas with epidemiological risk.