Fact 2: Primary vs Secondary HLH
When it comes to HLH, it’s not a one-size-fits-all situation. There are two distinct forms: primary and secondary. Primary HLH, also known as familial HLH, typically rears its head during infancy or childhood. This form is directly linked to certain genetic mutations that affect immune system function.
Primary HLH often runs in families, as the name suggests. When a child has primary HLH, there is a high probability that the syndrome is lurking in their genetic code. The disease results from a mutation in one of several genes that play a crucial role in the functioning of immune cells.
Secondary HLH, on the other hand, is more commonly diagnosed in adults and isn’t directly linked to a genetic mutation. Instead, secondary HLH arises as a result of some other condition or event that sets the immune system off balance. This could be an infection, a malignancy, or an autoimmune disease. (2)
