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Hemophagocytic Lymphohistiocytosis (Pediatric)

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What is hemophagocytic lymphohistiocytosis?

Hemphagocytic lymphohistocytosis is a rare disorder in which the immune system is overactive and attacks normal cells within the body, such as the liver, spleen, and the bone marrow or blood factory. There are two kinds of hemophagocytic lymphohistiocytosis:

  • Primary: hereditary condition
  • Secondary: when the condition develops after a severe infection, such as Epstein-Barr virus or some types of cancer.

How is hemophagocytic lymphohistiocytosis diagnosed?

Diagnosis of hemphagocytic lymphohistocytosis​​ entails:

  • History
  • Physical examination (i.e., fever and enlarged liver and spleen)
  • Complete blood count
  • Blood tests for liver function and chemical profile

What is the treatment for hemophagocytic lymphohistiocytosis?

Treatment of hemphagocytic lymphohistocytosis​​ includes:

  • Chemo-immunotherapy, which combines chemotherapy medicines with medicines that trigger immune response to attack the abnormal cells in more than way.
  • Hematopoietic stem cell transplantation