Concurrent hemophagocytic lymphohistiocytosis and thrombotic microangiopathy in a patient with Epstein-Barr virus infection and gastric adenocarcinoma: the puzzle started with microangiopathic hemolytic anemia.

Abstract

Malignancy-associated-hemophagocytic lymphohistiocytosis (HLH) is rare and often seen in high-grade lymphomas and acute leukemias; solid-tumor-associated HLH is extremely uncommon. The diagnosis of malignancy-associated-HLH remains challenging in clinical practices as it masquerades as and coexists with many other conditions. Here we presented a case with concurrent solid-tumor-associated HLH and thrombotic microangiopathy. The patient was an 80-year-old male with microangiopathic hemolytic anemia (MAHA), progressive bi-lineage cytopenia, and active Epstein-Barr virus (EBV) infection. Extensive lab works excluded all other alternative etiologies for MAHA but B12 deficiency, malignancy, and EBV infection. Concurrently, poorly differentiated gastric adenocarcinoma-associated HLH and thrombotic microangiopathy (TMA) were confirmed with extensive lab work. This patient passed away despite high-dose dexamethasone treatment. In the paper, we also discussed the possible pathophysiology of EBV infection in the development of MAHA and HLH and reviewed the treatment options for HLH and TMA.