A 13-year-old boy was referred to our haematology department with hyperthermia, headaches, confusion and vomiting, associated with hepatomegaly and haemorrhagic features (epistaxis and conjunctival haemorrhages). A full blood count showed pancytopenia: leucocyte count of 2·2 × 109/l, haemoglobin concentration of 100 g/l, platelet count of 28 × 109/l and reticulocyte count of 21 × 109/l. The peripheral blood film showed atypical lymphocytes and revealed ring forms of Plasmodium falciparum (left) with 2·5% parasitaemia. A diagnosis of airport malaria was made, as the patient had not travelled recently and lived near a military airport. He was treated with intravenous artenusate. Due to the persistence of cytopenias, bone marrow aspiration was performed and showed increased macrophages with haemophagocytosis of leucocytes and the presence of haemozoin in the cytoplasm (right). He fulfilled other macrophage activation syndrome (MAS) criteria, specifically a markedly elevated ferritin (11 840 µg/l) and hypertriglyceridaemia (3·88 g/l). By day 3, parasites were no longer detectable and the cytopenias and features of MAS started to resolve without the need for corticosteroid treatment. One platelet transfusion was needed. Treatment was continued with artemether and lumefantrine.
Macrophage activation syndrome/haemophagocytic lymphohistiocytosis is a hyperinflammatory condition with a high mortality rate. It can occur as a primary syndrome or more commonly secondary to an autoimmune, neoplastic or infection process, the latter including Epstein–Barr virus infection and also malaria. The pathogenetic mechanism proposed is an imbalance of pro- and anti-inflammatory cytokines leading to inappropriate immune stimulation and multi-organ dysfunction as a result of the hyperinflammatory response. The clinicopathological features of MAS and malaria can overlap. Clinicians therefore need to actively investigate for MAS when there is persistent fever or cytopenias despite appropriate anti-malarial therapy.
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