2019 Research Forum

Applicant: Mariam Abdelmisseh MD R3 Principal Investigator & Faculty Sponsor: Everardo Cobos MD

Hemophagocytic Lymphohistiocytosis Induced by Pregnancy and Postpartum

Mariam Abdelmisseh MD R3, Gowri Renganathan MD R2, Alyssa Targovnik MS IV, Kyle Foster MS IV, Amber Brewster MS IV, Everardo Cobos MD

INTRODUCTION Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially fatal immunopathology resulting in overproduction of cytokines and unregulated proliferation of macrophages ultimately leading to multi- organ injury. This condition is often triggered by a primary genetic disorder (commonly seen in the pediatric population), or by secondary triggers including but not limited to malignancy, rheumatologic process, or viral infection. Here we present a case of a hemophagocytic lymphohistiocytosis precipitated by pregnancy and the postpartum state.

PURPOSE To report a rare medical illness and increase awareness among medical providers since the medical illness is associated with increased mortality and morbidity.

DISCUSSION Patient is a 28-year-old G3P3 female, 22 days postpartum who presented to the ED with a one month history of persistent pyrexia and shortness of breath. Initial labs were significant for anemia, thrombocytopenia, transaminitis, and apparent hepatic dysfunction. CT abdomen additionally revealed hepatosplenomegaly. Due to unknown source of symptoms, a full sepsis workup was performed, however no source of infection was able to be found. She was started on broad-spectrum antibiotic therapy however hepatic dysfunction continued to deteriorate. Bone marrow biopsy was performed to rule out hematologic malignancy, results proved to be normal. Additionally, full infectious disease and rheumatologic workups were within normal limits. Further laboratory studies met diagnostic criteria of HLH as 5 of 8 criteria were met- in this particular case: elevated ferritin of 25074 ng/dL, elevated IL-2 of 16455, fever, hepatosplenomegaly, anemia, and thrombocytopenia. Subsequently, patient was started on corticosteroids and cyclosporine with resultant down-trending ferritin and IL-2 levels. Her hepatic function recovered with treatment as well. CONCLUSION Previously documented triggers of HLH such as those stated above did not seem to play a role in this case. This created a significant challenge in properly diagnosing and treating our patient. Extensive literature search revealed that there are only 14 documented cases of HLH associated with pregnancy and the post-partum state. We hope this case raises awareness of the importance of proper diagnosis and treatment, as patient are able to have significant response rates with proper therapy.

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