Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic Lymphohistiocytosis Latest News

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

About Hemophagocytic Lymphohistiocytosis 

• 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.
  • If both parents are genetic carriers of HLH, a child has a 25% chance of having the disease, a 25% chance of not having the disease, and a 50% chance of being a carrier.  
• 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

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Common Symptoms Of Hemophagocytic Lymphohistiocytosis

• The symptoms of HLH often resemble those of severe infections, making it difficult to diagnose in the early stages. 
• Symptoms may include: 
• Persistent high fever
  • Extreme tiredness and weakness
  • Enlarged liver and spleen
  • Swollen lymph nodes
  • Skin rash
  • Easy bruising or bleeding
  • Pale skin due to anemia
  • Low blood cell counts
  • Yellowing of the skin or eyes (jaundice)
  • Confusion, seizures, or other neurological symptoms in severe cases

Hemophagocytic Lymphohistiocytosis 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.

News: NDTV