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Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic lymphohistiocytosis (HLH) is a non-malignant but life threatening disease. Every year, approximately 4-6 children in the Netherlands are diagnosed with HLH. HLH can occur at any age, often in young babies even.

The brochure entitled Hemophagocytic lymphohistiocytosis contains comprehensive information about this disease. The most important information is provided below.

Causes

HLH is caused by a dysregulation of the immune system. The immune cells do not work properly and so, in an attempt to get the immune system back up, they divide uncontrollably. This causes an enormous secretion of inflammatory proteins. There are hereditary (primary) forms of HLH that often occur at a young age, but there are also secondary forms. The latter are a side effect of an infection, a rheumatological disease or cancer. The immune system has got out of control and is no longer properly regulated. The immune cells will continue to fuel HLH, particularly if the infection or the cancer is still present in the body.

Symptoms

Children with HLH are often very sick, have a high fever that does not respond to antibiotics, swollen lymph nodes and a lack of healthy blood cells. Other symptoms include skin rash, an enlarged liver and spleen and sometimes neurological symptoms.

How is HLH diagnosed?

The symptoms and the results of a blood test combined are often sufficient to make the diagnosis. Treatment can then commence without delay and before your child gets any sicker. It is also checked whether heredity figures in your child's disease.

Treatment

Treatment consists of a combination of drugs that suppress inflammation (immunosuppression) and chemotherapy. A hereditary form of HLH requires a stem cell transplant with donor stem cells once the disease is stable. A stem cell transplant is also required when a non-hereditary form repeatedly causes symptoms.

Chance of recovery

Recovery strongly depends on how the disease can be brought under control.

Questions?

Children and teenagers with HLH are treated in the hematology-oncology department.

Telephone 088-9727272