Poster Presentation Society of Obstetric Medicine of Australia and New Zealand ASM 2026

HLH-like Systemic Autoinflammatory Syndrome with Necrotising Lymphadenitis in Early Pregnancy: A Case Report  (#138)

Nik Nur Iman Nik Ghazali 1 , Soe Min Myat 1 , Ling Li 1 , Sharath Heggodu Devappa 1
  1. Endocrinology and Obstetric Medicine, Cairns Base Hospital, Cairns, QLD, Australia

Introduction: 
Pyrexia of unknown origin in pregnancy with cytopenias, hyperferritinaemia, and lymphadenopathy presents a significant diagnostic challenge. Kikuchi–Fujimoto disease (KFD), haemophagocytic lymphohistiocytosis (HLH), infection, malignancy, and systemic autoinflammatory disorders may present with overlapping features. Pregnancy further complicates interpretation of inflammatory markers and disease severity, making early recognition essential for timely treatment.

 

Case: 
A 36-year-old primigravida presented at 9 weeks’ gestation with a four-week history of fever, drenching night sweats, profound fatigue, weight loss, periorbital swelling and scalp papular lesions. Investigations demonstrated pancytopenia, hepatitis, marked hyperferritinaemia (>3000 µg/L) and multifocal lymphadenopathy. Extensive infectious investigations were unrevealing despite several cross-reactive positive serologies. Autoimmune screening was negative. Lymph node biopsy demonstrated necrotising lymphadenitis without evidence of malignancy. Although HLH was considered, diagnostic criteria were not fulfilled. Progressive maternal deterioration resulted in surgical termination of pregnancy at 12 weeks’ gestation. Treatment with anakinra produced rapid clinical and biochemical improvement, with resolution of fevers, cytopenias, hepatitis, lymphadenopathy and skin lesions. 

 

Discussion: 
KFD is a rare necrotising lymphadenitis that predominantly affects young women. KFD has been reported in association with HLH, a life-threatening hyperinflammatory syndrome characterised by fever, cytopenias, hepatitis and hyperferritinaemia. Although HLH criteria were not fulfilled in this case, the clinical phenotype was consistent with HLH-like inflammatory process. The marked response to interleukin-1 blockade supports a cytokine-driven autoinflammatory mechanism. 

 

Conclusion: 
This case highlights a pregnancy-associated overlap of KFD and HLH-spectrum inflammation with dramatic response to IL-1 blockade. Recognition of autoinflammatory causes of PUO in pregnancy may enable earlier targeted therapy and improved outcomes. 

  1. Altuntas F, Sari I, Canoz O, Yildiz O, Eser B, Cetin M, Unal A. Kikuchi-Fujimoto disease: a rare but important cause of fever and lymphadenopathy in pregnant women. Am J Hematol. 2006 Feb;81(2):118-20.
  2. Wilson-Morkeh H, Frise C, Youngstein T. Haemophagocytic lymphohistiocytosis in pregnancy. Obstet Med. 2022 Jun;15(2):79-90.
  3. Markouli M, Diamantopoulos P, Chalioti A, Lontou S, Mantzourani M. Kikuchi-Fujimoto Disease and Hemophagocytic Lymphohistiocytosis: A Rare Combination of Two Rare Diseases. Mediterr J Hematol Infect Dis. 2026 Jan 1;18(1):e2026006.
  4. Ichikawa G, Negishi Y, Chishima F, Suzuki S. Kikuchi–Fujimoto disease during early pregnancy. J Obstet Gynaecol Res. 2024; 50(6): 1056–1060.