Abstract
Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease. It is characterized by impaired natural killer cell and cytotoxic lymphocyte function leading to dysregulated macrophage phagocytosis of erythrocytes, leukocytes and platelets. Coupled with a massive release of cytokines this rapidly leads to multi-organ failure and mortality if not treated promptly. Case presentation We report a case of pregnancy-associated, treatment-resistant HLH with no identified risk factors. Conclusion It is likely that HLH is an unrecognized cause of intractable fever and disseminated intravascular coagulation (DIC) in young pregnant women and it should be included in the differential diagnosis when patients do not respond to typical therapies. High serum ferritin should further prompt consideration of this diagnosis. This rare disease is often not thought of and many gynecologist obstetricians have never heard of it, making this case report of essential importance to reignite the interest in HLH, particularly when it occurs during pregnancy.
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