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

Roger B RathnaArkadeep DhaliAthul Kurien VargheseChaitanya H Balakrishnan
Published in: BMJ case reports (2022)
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.
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