S. Wautier MD, C. Mahiat MD, T. Connerotte MD, PhD, N. Whenham MD, R. Poncin MD, L. Duck MD
Haemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease characterised by cytotoxic immune deregulation leading to hypercytokinaemia and macro-phage activation. Given the high mortality rate, HLH must be suspected in nonspecific situations and promptly confirmed based on 2004-HLH criteria. In adult patients, HLH is usually secondary to infection, malignancy or underlying autoimmune diseases. As treatment, paediatric protocol consists of immuno-therapy and chemotherapy, followed by a haematopoietic stem cell transplant (HSCT). In adults, individualised modified treatment is recommended because of the variable cause and severity of situation. Corticosteroids are the mainstay. We report the case of a patient with breast cancer, for whom we made the diagnosis of HLH during her neoadjuvant chemotherapy based on the 2004-HLH criteria. A viral aetiology was suspected with reactive lymphocytes found on the blood smear, and we promptly initiate treatment of dexamethasone. Our patient fully recovered both from her HLH and breast cancer.