An interesting cause of liver failure: Secondary Haemophagocytic Lymphohistiocytosis (HLH) – A Case Series
Thomas Garvey, Jon McKee, Johnny Cash, Ian Cadden
Liver Unit, Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA
Hemophagocytic lymphohistiocytosis (HLH) is a rare aggressive disease, which is potentially life threatening, in which certain white blood cells (histiocytes and lymphocytes) build up in organs including the skin, spleen, and liver, and destroy other blood cells. This usually causes fever and damages the liver and spleen and can result in enlargement of these organs. HLH may be inherited or acquired. Secondary HLH (acquired form) occurs after strong immunologic activation, such as that which can occur with systemic infection (mostly viral infections), immunodeficiency, autoimmune diseases or underlying malignancy.
We present two cases of patients who presented to district general hospitals (DGH) with pyrexia, jaundice, malaise and deranged liver function tests in conjunction with high ferritin levels and cytopenias. Both patients were transferred to the same regional liver unit due to worsening liver function. Both underwent investigations showing findings indicating a haemophagocytic syndrome. Bone marrow biopsies confirmed HLH in both patients. One patient had a natural killer (NK) cell leukaemia as the precipitant whilst the other had Epstein Barr Virus (EBV) – associated HLH. The aim of this case series is to increase awareness for early detection for patients with secondary HLH, especially those presenting with fulminant hepatic failure (FHF).
Whilst we were unable to alter the course of the disease in the patient with NK cell leukaemia, we highlight the case of the 20 year old female with EBV-associated HLH, who regained normal liver function after treatment with dexamethasone and etoposide.
HLH should be considered in the differential diagnosis of FHF, especially in patients presenting with clinical signs of acute liver failure accompanied by cytopenias, high ferritin levels and unexplained prolonged fever. HLH can lead to severe liver damage and awareness and early recognition of the clinical symptoms and diagnostic criteria is important to start potentially life-saving therapy, and aim to regain normal liver function.