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Histiocytosis
Disease information
Overview
Histiocytosis is a condition where certain immune cells called histiocytes start growing out of control. These overactive cells can start to absorb other cells in the body and cause an intense immune reaction, which can lead to various health issues.
Doctors classify histiocytosis based on whether a specific type of histiocyte known as a Langerhans cell is involved and how severe the condition is. Langerhans cell histiocytosis is a rare disease where there's an overproduction of Langerhans cells, which are white blood cells that have a role in the immune system, like eating up harmful cells and viruses. When these cells go rogue, they can invade and damage different parts of the body, often starting with the bones. But they can also affect the skin, lungs, and bone marrow. There's also a related condition called hemophagocytic lymphohistiocytosis (HLH), which is all about problems with macrophages, another kind of immune cell. HLH can either be something you're born with (primary HLH) or something that develops because of another disease or condition (secondary HLH).
Causes and Symptoms
The exact cause of conditions like Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis (HLH) is still a bit of a mystery, but in some cases, doctors have found links to genetic irregularities. With Langerhans cell histiocytosis, even though the cells look the same under the microscope, the symptoms and how serious the disease is can vary a lot, depending on which organ is affected. HLH is usually triggered by problems with certain immune cells – specifically T cells or natural killer (NK) cells – which then go into overdrive, pumping out too many inflammatory substances called cytokines, and these are what cause the symptoms of the disease.
Treatment
For Langerhans cell histiocytosis, the treatment plan can vary quite a bit. It might be as simple as scraping away the affected area (curettage) or as complex as surgery to remove it. Doctors also might use chemotherapy or radiation therapy to target the disease. And in serious cases where the outlook isn't good, a treatment option is a hematopoietic stem cell transplantation, which is a way to try to give the patient a new immune system to fight the disease.
When it comes to hemophagocytic lymphohistiocytosis, the main aim is to save lives because this can be a deadly condition. Treatment usually starts right away to stop the disease from getting worse, often with chemotherapy drugs like etoposide and steroids to tamp down the inflammation. If the disease doesn't get better with initial treatment, or if it comes back, then doctors may consider a hematopoietic stem cell transplantation as a next step.