Rare association of haemophagocytic lymphohistiocytosis (HLH) and antiphospholipid syndrome (APS) in postpartum period: a considerable overlap?

Haemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening disease of disproportionate immune activation. We present a case of a 25-year-old woman who is postnatal day 26 status post lower segment caesarean section who came with fever, breathlessness and abdominal pain in shock in the background of a malarial infection 3 days ago, which was initially evaluated on the lines of fever with multiple organ dysfunction syndrome. On further evaluation turned out to fulfil the criteria for secondary HLH seemingly triggered by an infectious cause or an autoimmune phenomenon. On further assessment, she was found to be positive for antiphospholipid antibodies which forced other possible diagnoses such as catastrophic antiphospholipid antibody syndrome into contention.

Automated summary

HLH is an aggressive and life-threatening disease characterized by disproportionate immune activation. This case may be a secondary HLH triggered by an infection or autoimmune response, with the patient also testing positive for antiphospholipid antibodies.