Peripartum COVID-19 & hemophagocytic lymphohistiocytosis: a case report

Abstract

Objectives: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of hypercytokinemia and immune dysregulation. Most commonly diagnosed in the pediatric population due to genetic predisposition, the condition can manifest in the adult population secondary to an immune dysregulating event, such as infection, malignancy, rheumatologic disorders, immunodeficiency, and checkpoint inhibitors. The presentation and diagnosis during pregnancy are extremely rare and elusive. We present a case of secondary HLH during the peripartum period, urging obstetrical providers to keep the condition as part of their differential diagnosis.

Case presentation: A 20-year-old Gravida 1, with a past medical history significant for non-alcoholic hepatosteatosis and morbid obesity, presented multiple times to the emergency department in the third trimester with liver function test derangements and vague complaints of subjective fevers and fatigue. She eventually tested positive for COVID-19. Two weeks after the initial presentation, she went into spontaneous preterm labor and delivered. Postpartum, her liver dysfunction worsened in association with high fevers and persistent tachycardia. After an extensive workup failed to reveal an etiology, HLH was suspected. Labs were sent for confirmation, and she was initiated on pulse-dose steroids. However, the patient acutely decompensated and succumbed to the disease. Several days later, labs resulted, confirming the diagnosis of HLH.

Conclusions: In peripartum patients presenting with severe derangements in liver function tests and vague symptoms with undulating episodes of pyrexia, HLH should be considered early as part of the differential diagnosis. This is particularly true when antibiotics or postpartum status fail to alleviate the symptomatology or improve the clinical course.

Keywords: COVID-19; hemophagocytic lymphohistiocytosis; pregnancy; puerperal.