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Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic Lymphohistiocytosis (HLH):

Former Afghanistan fast bowler Shapoor Zadran died at the age of 38 recently after battling Hemophagocytic Lymphohistiocytosis (HLH), a rare immune system disorder.

Hemophagocytic Lymphohistiocytosis (HLH):

  • It is a rare but serious condition in which the body’s immune system becomes overactive.
  • Instead of protecting the body from infections, the immune system starts attacking healthy tissues and organs.
  • This causes severe inflammation that can affect the liver, spleen, bone marrow, brain, and other parts of the body.
  • The condition can become life-threatening if left untreated.
  • HLH can occur for different reasons. It is mainly divided into two types:
    • Primary (Familial) HLH:
      • This type is caused by genetic changes passed down from parents.
      • It usually appears during infancy or early childhood, although it can sometimes develop later in life.
    • Secondary (Acquired) HLH:
      • This type develops due to medical conditions that trigger the immune system.
  • Common triggers include:
    • Viral infections such as Epstein-Barr virus (EBV) or cytomegalovirus (CMV)
    • Certain bacterial, fungal, or parasitic infections
    • Autoimmune diseases like lupus or rheumatoid arthritis
    • Blood cancers such as lymphoma or leukemia
    • A weakened immune system due to certain medications or medical conditions
  • Treatment:
    • HLH treatment depends on the cause, the patient’s age when the disease starts, and how severe the disease is.
    • Chemotherapy and cancer drugs, immunotherapy, steroids that fight inflammation, antibiotics, and antiviral drugs are all mentioned as part of the treatment protocol.