Haemophagocytic lymphohistiocytosis (HLH): a rare but potentially fatal association withPlasmodium vivaxmalaria

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that is caused by an abnormal activation of the immune system. It can present as the primary syndrome or occur secondary to a variety of conditions such as malignancy, autoimmune diseases and infections. We present a case of a man who developed HLH secondary to Plasmodium vivax infection. He presented with symptoms of fever, chills and myalgias. Physical examination revealed significant hepatosplenomegaly. The presence of pancytopaenia, elevated ferritin levels and haemophagocytosis on bone marrow biopsy confirmed the diagnosis of HLH (based on HLH-2004 criteria). There was a significant improvement after the initiation of intravenous antimalarials. No relapses were documented on follow-up. It is imperative that physicians should promptly recognise and treat this rare condition, as a timely intervention can be lifesaving.

Takotsubo cardiomyopathy secondary to haemophagocytic lymphohistiocytosis in HIV patients: a comprehensive review

“…However, the mechanism of pathogenesis by these markers is unknown 1. This mechanism explains the multisystem organ failure, high morbidity and mortality associated with HLH 1. Our patient also had classic multisystem involvement with the unique cardiac involvement, causing globular hypokinesis and presenting as TCM.…”

“…Its worldwide incidence is not well reported in adults. However, about 1.2 million children are affected by HLH each year 1. Traditionally, HLH was characterised into primary and secondary HLH, primary being due to genetic causes which usually occurs at early age and is rare later in life.…”

“…HLH is a disease of hyperinflammation characterised by uncontrolled proliferation of activated lymphocytes and macrophages that secrete excessive amounts of inflammatory cytokines and by the inability of the natural killer cells and cytotoxic T-cells to downregulate the immune response 1. This leads to immune dysregulation by inducing apoptosis in themselves and other elements at the forefront of the immune response such as macrophages after a threat is neutralised.…”

“…IL-2 and its receptor CD-25 have a diagnostic role in the HLH criteria and has been an important indicator of increased T-cell activity. However, the mechanism of pathogenesis by these markers is unknown 1. This mechanism explains the multisystem organ failure, high morbidity and mortality associated with HLH 1.…”

“…HLH usually has non-specific presenting features but fever and high ferritin are present in almost all patients, while other integral features are hepatosplenomegaly, cytopaenia and high LDH1 1. Diagnosis is made using the criteria devised by HLH-2004 trial as shown in box 1.…”