What causes HLH disease?

Children usually inherit the disease. In adults, many different conditions, including infections and cancer, can cause HLH. If you have HLH, your body’s defense system, called your immune system, does not work normally. Certain white blood cells — histiocytes and lymphocytes — attack your other blood cells.

Is HLH a form of leukemia?

In patients with acute myeloid leukemia (AML), HLH has been occasionally described in case-reports. AML patients may be prone to develop HLH due to their disease- and/or treatment-related impaired immune response and their high susceptibility to severe infections, which act as triggering factors.

What is MAS disease?

Background. Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages.

What does Hemophagocytic mean?

(HEE-moh-FA-goh-SIH-tik LIM-foh-HIS-tee-oh-sy-TOH-sis) A rare disorder in which histiocytes and lymphocytes (types of white blood cells) build up in organs including the skin, spleen, and liver, and destroy other blood cells.

What is hemophagocytic lymphohistiocytosis?

Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues.

What is virus-associated hemophagocytic syndrome (Vas)?

Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer. 1979 Sep;44(3):993–1002.

What is the pathophysiology of Secondary haemophagocytic lymphohistiocytosis?

Secondary haemophagocytic lymphohistiocytosis (acquired haemophagocytic lymphohistiocytosis) occurs after strong immunologic activation, such as that which can occur with systemic infection, immunodeficiency, or underlying malignancy.

What are the signs and symptoms of hemophagocytosis?

Hemophagocytosis (the destruction of certain types of blood cells by histiocytes) on bone marrow, spleen or lymph nodebiopsy Decreased or absent NK cell activity High levels of ferritin in the blood Elevated blood levels of CD25 (a measure of prolonged immune cell activation).