Malignancy-induced hemophagocytic lymphohistiocytosis in a case of signet ring cell carcinoma of the stomach.

ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening condition associated with high mortality and can be classified into primary (familial) and secondary HLH. Primary HLH is an inherited, autosomal recessive disorder associated with defects in perforin function, whereas secondary HLH is associated with infections, especially Epstein-Barr virus infection, malignancies, and autoimmune disorders. Patients with malignancy-associated secondary HLH experience symptoms that overlap with those described for other HLH types, which is associated with an increased incidence of misdiagnosis and mortality. Here, we report the case of a patient with secondary HLH associated with a solid malignancy (signet-ring cell carcinoma of the stomach).