Hemophagocytic Lymphohistiocytosis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder characterized by uncontrolled inflammation due to defective immune response. It may be familial or acquired, but both share a common feature of threatening the life of a patient and may lead to death unless treated by appropriate treatment. Here in we report a case of adult HLH.

Fatal diagnosis of hemophagocytic lymphohistiocytosis in a patient with a history of human immunodeficiency virus, Hodgkin’s lymphoma, and Epstein–Barr virus

“…In the United States, the prevalence is estimated to be 1/100,000 live births [1]. Primary HLH is familial or genetic in origin, usually presents in infants or children, and is usually fatal within two months without treatment [2]. Secondary HLH, which was seen in our patient, is secondary to an underlying illness such as malignancy, infection, or autoimmune diseases.…”

“…It usually presents in infants and young children and has a low likelihood of survival. Secondary HLH is acquired from an underlying illness [2]. Common triggers for acquired HLH are malignancy, especially hematologic, and infections.…”

Successful diagnosis and treatment of scrub typhus associated with haemophagocytic lymphohistiocytosis and multiple organ dysfunction syndrome: A case report and literature review

“…HLH is a syndrome of immune system activation which leads to phagocytosis of blood cells and proliferation of histiocytes in solid organs. Scrub typhus invades endothelial cells and macrophages, activating various chemokine genes [ 1 , 2 , 28 , 29 , 30 , 31 , 32 , 33 ]. These inflammatory cytokines are responsible for HLH and MODS in patients with severe infections [ 1 , 2 , 29 , 30 , 34 , 35 ].…”

“…Scrub typhus invades endothelial cells and macrophages, activating various chemokine genes [ 1 , 2 , 28 , 29 , 30 , 31 , 32 , 33 ]. These inflammatory cytokines are responsible for HLH and MODS in patients with severe infections [ 1 , 2 , 29 , 30 , 34 , 35 ]. The HLH-2004 criteria are used to identify HLH, which are summarized in Table 3 [ 36 ].…”

Clinical characteristics and effective treatments of scrub typhus-associated hemophagocytic lymphohistiocytosis in children

“…However, some patients may experience severe or fatal events, such as acute renal failure, respiratory distress and acute liver failure [4]. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can rapidly deteriorate and cause multiple organ failure and even death [5], [6]. HLH can be triggered by rickettsial diseases and other zoonoses [7], [8].…”