HLH stands for Hemophagocytic Lymphohistiocytosis, which is a rare and potentially life-threatening disorder that affects the immune system. It is characterized by an excessive activation and proliferation of certain immune cells, specifically histiocytes and lymphocytes, leading to inflammation and organ damage.
In HLH, the immune system becomes overactive, causing the immune cells to attack and destroy healthy tissues and organs. This abnormal immune response can occur secondary to various underlying conditions, including viral, bacterial, or fungal infections, autoimmune disorders, or certain genetic mutations. It is more commonly seen in infants and young children, but it can occur in people of any age.
Symptoms of HLH can vary widely but often include persistent high fever, enlarged liver and spleen, skin rash, swollen lymph nodes, and low blood cell counts. Other manifestations may include neurological abnormalities, respiratory problems, and organ failure.
Prompt diagnosis and treatment of HLH are crucial to improve outcomes. Treatment typically involves a combination of immunosuppressive drugs, such as corticosteroids and immunomodulatory agents, to suppress the overactive immune response. Additionally, patients may require supportive therapies, such as blood transfusions or stem cell transplantation, to address complications and restore immune function.
Early recognition and management of HLH are essential for preventing further damage and improving the chances of survival.
