Hemophagocytic lymphohistiocytosis is an aggressive, life-threatening and often fatal disease, even with appropriate treatment, in which over-activation of the immune system leads to haematopoietic cell death. Triggering factors include malignant diseases, infections and presence of acquired and inherited immunodeficiency. It usually presents as an acute febrile illness with multi-organ failure. The most common clinical manifestations include fever, hepatosplenomegaly, cytopenia, liver function and coagulation abnormalities, elevated triglyceride, ferritin and lactate dehydrogenase levels. In addition to the above, diagnostic criteria include bone marrow biopsy and various immunological tests.
Diagnostic and treatment options are presented through a case of a patient with aggressive cytotoxic T-cell lymphoma. Following several lines polychemotherapy, she was admitted urgently with fever and liver failure. Her laboratory results showed elevated liver function, inflammatory parameters, lactate dehydrogenase, triglyceride and ferritin levels, impaired renal function, anemia and thrombocytopenia. Abdominal ultrasound scan showed hepatosplenomegaly. The result of bone marrow trephine biopsy confirmed the suspected diagnosis of haemophagocytic lymphohistiocytosis and showed the relapse of lymphoma. Firstly, she was given intravenous steroid and high dose intravenous immunoglobulin. After that, in light of the supporting bone trephine biopsy report, etoposide was administered, which resulted in significant but transient improvement in clinical symptoms and laboratory parameters.
Haemophagocytic lymphohistiocytosis is a life-threatening condition that can make the difficult fate of patients with T-cell lymphoma even worse. Early diagnosis and appropriate treatment of the underlying cause can delay the development of multi-organ failure, while the prognosis is determined by the clinical course of the lymphoma.