Infection-associated haemophagocytic syndrome associated with recurrent acute myeloid leukaemia/myelodysplastic syndrome: an autopsy case

Infection-associated haemophagocytic syndrome (IAHS) is an uncommon disorder usually occurring in immunocompromised patients. IAHS is a systemic reactive proliferation of phagocytic histiocytes induced by an infection usually of viral origin.¹ We report a fatal case of IAHS arising in a patient with recurrent acute myeloid leukaemia (AML)/myelodysplastic syndrome (MDS). The diagnosis of IAHS was made after autopsy and histological evaluation of tissue.

CLINICAL HISTORY

A 60-year-old man with a history of acute myelogenous leukaemia (M4 myelomonocytic leukaemia) status post complete remission after chemotherapy 5 years ago was admitted for progressive fatigue and pancytopenia. He was in good health until 1 year ago when he started to develop macrocytic anaemia and thrombocytopenia. A bone marrow biopsy at that time showed changes consistent with myelodysplastic syndrome (refractory anaemia). Over the next few months, he developed a progressive pancytopenia but did not come in for evaluation. He was admitted to his local hospital 1 month before being transferred to our institution for progressive fatigue and pancytopenia (white cells 5300/μl, haemoglobin b 4.1 g/dl and platelets 22 0000/μl). The patient had intermittent swelling of different parts of the body, including the leg and jaws. The hospital course was dominated by unexplained daily nocturnal fevers with temperatures rising up to 102°F. The patient was transferred to our institution to further investigate the unexplained fever and to manage …