A Rare and Fatal Case of Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis

Hemophagocytic syndrome in patients with long-term stable pulmonary sarcoidosis with progressive spleen and bone marrow lesion

“…The present case of HPS occurred 17 years after the diagnosis of sarcoidosis, and there is also one previous case of HPS after a 30-year period [8]. Of the eleven reported cases, five had viral or tuberculous infections, and six had HPS without a specific trigger [3]. In the present case, urinary tract infection was suspected, but no other infections could be identified, although we searched for cytomegalovirus, Epstein-Barr virus, and M.…”

“…We found 11 single-case reports of reactive hemophagocytic syndrome due to sarcoidosis. Of these eleven featured patients, only three patients survived, with the mortality rate of 73% [3].…”

“…The mechanism of pancytopenia in patients with sarcoidosis is reported to be impairment of hematopoiesis due to granuloma replacement in the bone marrow, peripheral blood cell depletion due to migration of cells to the site of inflammation, blood cell destruction due to increased splenic function, or immunological destruction [2]. Reactive hemophagocytic syndrome (HPS) is another cause of pancytopenia, with sarcoidosis as the underlying disease [3].…”

“…Reactive HPS has a high mortality rate of 50-75% and requires immediate treatment with steroid pulses, immunosuppressive agents, and biologics [3]. Consideration and treatment of HPS soon after onset is important for treatment to be successful.…”