Acquired Hemophagocytic Lymphohistiocytosis (HLH)

What is acquired hemophagocytic lymphohistiocytosis (HLH)?

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune system disease. It occurs more often in babies and young children. But it can also occur in adults. Children may inherit the disease through genes. This is called familial HLH. But in adults, many conditions can cause HLH. This is called acquired HLH. It can be caused by some infections, cancer, and autoimmune diseases.

If you have acquired HLH, your body's immune system does not work normally. Some white blood cells called histiocytes and lymphocytes attack your other blood cells. Abnormal blood cells then build up in your spleen and liver. This causes your spleen and liver to enlarge. HLH is a life-threatening condition. It can cause death in weeks or months if not treated. It may also cause death in months despite treatment.

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What causes acquired HLH?

HLH is a rare disease, and researchers are still learning what causes it. There are 2 types of HLH: familial and acquired. Familial HLH is passed from parents to children. Acquired HLH in adults can be caused by:

  • Viral infections, most often Epstein-Barr virus
  • Other infections from bacteria or fungi
  • Some types of cancer, such as T-cell lymphoma
  • Autoimmune diseases
  • Medicines that suppress the immune system

If your acquired HLH is caused by a virus, you may have another genetic disease called X-linked lymphoproliferative disease (XLP).

Who is at risk for acquired HLH? 

You are more at risk for acquired HLH if you have any of the following:

  • Epstein-Barr virus
  • Cancer
  • Autoimmune disease
  • Taking medicine to suppress the immune system

What are the symptoms of acquired HLH?

The most common symptoms of acquired HLH are:

  • Fever
  • Enlarged spleen

Other symptoms can include:

  • Enlarged liver
  • Swollen lymph nodes
  • Skin rash
  • Yellow color of your skin and eyes (jaundice)
  • Lung problems such as coughing and trouble breathing
  • Digestive problems such as stomachache, vomiting, and diarrhea
  • Nervous system problems such as headaches, trouble walking, vision disturbances, and weakness

The symptoms of acquired HLH can look like other health conditions. See your healthcare provider for a diagnosis.

How is acquired HLH diagnosed?

Your healthcare provider will ask about your symptoms and health history. The provider may also ask about your family’s health history. They will give you a physical exam. The physical exam may include feeling your belly where your liver and spleen are. You will also have blood tests. These are done to look for:

  • Low levels of white blood cells called natural killer cells
  • Low levels of other white blood cells, red blood cells, and platelets
  • High levels of fats in your blood called triglycerides
  • Low levels of fibrinogen, a protein needed for clotting
  • High levels of ferritin, a protein that stores iron
  • High levels of a substance called CD25, which increases in your blood when your immune system is active
  • Signs of infection
  • Genetic changes

You will also have a bone marrow biopsy. Bone marrow is soft tissue in the center of some large bones. Blood cells are made in bone marrow. For this test, a sample of your bone marrow is taken with a needle. The tissue is looked at in a lab with a microscope.

How is acquired HLH treated?

Treatment will depend on your symptoms, your age, and your general health. It will also depend on how severe the condition is.

Acquired HLH may go away when your healthcare provider finds and treats the cause, such as an infection. In other cases, more treatment is needed. Treatment for acquired HLH that doesn’t go away may include:

  • Cancer medicines (chemotherapy)
  • Medicines that affect your immune system (immunotherapy)
  • Medicines that fight inflammation (steroids)
  • Antibiotic medicines
  • Antiviral medicines

If medicine treatments don’t work, your healthcare provider may do a stem cell transplant. In this procedure, your diseased bone marrow cells are replaced by healthy bone marrow cells from a donor. Stem cell transplant can cure HLH in most cases.

Talk with your healthcare providers about the risks, benefits, and possible side effects of all treatments.

What are possible complications of acquired HLH?

HLH is a life-threatening condition. It can cause death in weeks or months if not treated. It may also cause death in months even with treatment.

Can acquired HLH be prevented?

Researchers don’t know how to prevent acquired HLH.

Online Medical Reviewer: Todd Gersten MD

Online Medical Reviewer: Raymond Turley Jr PA-C

Online Medical Reviewer: L Renee Watson MSN RN

Date Last Reviewed: 1/1/2021

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