Secondary hemophagocytic lymphohistiocytosis (HLH) occurs in association with infections, autoimmune disorder or malignancy. Only few studies reported cases of infection-associated HLH (I-HLH). We aimed to study the clinical, laboratory and therapeutic features of I-HLH among children and adults.
Material and methods:
We conducted a retrospective study including all patients hospitalized in Pediatric and infectious diseases department with confirmed diagnosis of I-HLH between 2011 and 2017.
We enrolled 19 cases with I-HLH, among whom 11 cases were males (57.8%). We identified 15 children and 4 adults. The median age was 11 years [1-65 years]. Physical examination showed splenomegaly in 15 cases (78.9%) and hepatomegaly in 10 cases (52.6%). Regarding laboratory examinations, anemia and elevated ferritin levels were noted in all cases. Bone marrow aspiration showed images of haemophagocytosis in all cases. Bacterial infection was noted in 10 cases (52.6%) and parasitic infection in 5 cases (26.3%). All patients received symptomatic and/or etiological treatment. Specific treatment with corticosteroids and human immunoglobulins were used in 7 cases (36.8%). Ten patients were dead (52.6%) due to multiple organ failure.
Hemophagocytic lymphohistiocytosis was a life-threatening disease. The prognosis depends on the underlying etiology, but remains poor. Prompt diagnosis and treatment are crucial in order to improve the outcome.