Table of Contents
What is the meaning of HLH?
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that usually occurs in infants and young children. It may also occur in adults. Children usually inherit the disease. In adults, many different conditions, including infections and cancer, can cause HLH.
What is the survival rate for HLH?
More recent studies have shown that the HLH-94 protocol resulted in an overall survival rate of 55\%. Success or failure of an allogeneic BMT is the most important long-term prognostic factor. Unfortunately, many cases are diagnosed late in the course of the disease, after irreversible damage has occurred.
What causes hemophagocytic lymphohistiocytosis?
Conditions that can lead to secondary hemophagocytic lymphohistiocytosis include viral infections especially Epstein-Barr virus, other infections including bacterial, viral and fungal infections, a weakened or depressed immune system, autoimmune diseases, autoinflammatory diseases, rheumatological diseases such as …
What is HLS disease?
Hydrolethalus (HLS) is a severe fetal malformation syndrome characterized by craniofacial dysmorphic features, central nervous system, cardiac, respiratory tract and limb abnormalities.
Is secondary HLH fatal?
Hemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome that can be fatal. This syndrome can sometimes occur in normal people with medical problems that can cause a strong activation of the immune system, such as infection or cancer. HLH in these settings is called Secondary HLH.
Can acquired HLH be cured?
Stem cell transplant can cure HLH in most cases. Talk with your healthcare providers about the risks, benefits, and possible side effects of all treatments.
How is hemophagocytic lymphohistiocytosis diagnosed?
HLH can be diagnosed only with the proper tests. Blood tests include blood cell counts, liver function, infection studies, and markers of immune system activation such as ferritin and soluble IL-2 receptor levels. A bone marrow aspirate and biopsy may be performed to look for microscopic evidence of hemophagocytosis.
What is HLH in adults?
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that usually occurs in infants and young children. It may also occur in adults. Children usually inherit the disease. In adults, many different conditions, including infections and cancer, can cause HLH.
Is HLH cancer?
HLH is a rare condition where the body produces too many white blood cells called lymphocytes and histiocytes. If it is not treated, these extra cells collect in organs in the body and damage them. HLH is not cancer, but it is similar to cancer and usually treated by cancer specialists.
What is HLH blood disease?
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of the immune system. It more often occurs in babies and young children. But it can also occur in adults. In adults, many conditions can cause HLH. This is known as acquired HLH. It can be caused by some infections, cancer, and autoimmune diseases.