recurrent Hemophagocytic Lymphohistiocytosis

What is Recurrent Hemophagocytic Lymphohistiocytosis?

Recurrent Hemophagocytic Lymphohistiocytosis (or "RLH") is an autoimmune disorder characterized by an overproduction of certain immune cells. This leads to an overactive immune response, resulting in inflammation and tissue damage throughout the body. RLH can affect people of all ages, but is more commonly seen in children. RLH is most commonly caused by a genetic mutation, but can also be triggered by infections, medications, or underlying medical conditions. Without prompt and appropriate treatment, RLH can be fatal.

Symptoms of Recurrent Hemophagocytic Lymphohistiocytosis

Symptoms of RLH can vary, and may include:

  • Fever
  • Chills
  • Rashes
  • Joint pain
  • Enlarged spleen or liver
  • Lethargy
  • Weight loss
  • Generalized weakness

Diagnosis of Recurrent Hemophagocytic Lymphohistiocytosis

The diagnosis of RLH is based on a physical exam and a blood test. During the physical exam, the doctor will check for signs of swelling or tenderness in the liver or spleen. Blood tests may include a complete blood count (CBC), which can reveal anemia and low platelet count, as well as evidence of an overactive immune system. A bone marrow biopsy may also be done to confirm the diagnosis.

Treatment of Recurrent Hemophagocytic Lymphohistiocytosis

Treatment of RLH depends on the underlying cause. In cases where a genetic mutation is present, treatment may include chemotherapy, immunosuppressive medications, or therapy to correct the mutation. For infections or other triggering factors, treatment may include antibiotics or medications to suppress the immune system. In general, the goal of treatment is to reduce inflammation, prevent tissue damage, and prevent complications.