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What Is Chronic Graft-Versus-Host Disease? | BY HEIDI


 

Graft-versus-host disease (GvHD) is a condition in which the immune cells of a transplanted organ (graft) attack the cells of the person who received the transplant (the host). It can occur after a bone marrow transplant or a stem cell transplant.



These procedures treat certain types of diseases and cancer, especially cancer the involves the blood cells. 



Types of Graft-Versus-Host Disease 

Graft-versus-host disease can be acute or chronic. These are distinguished based on how long it takes for symptoms to occur after the transplant:1


Acute graft-versus-host disease occurs within 100 days of the transplant.

Chronic graft-versus-host disease is defined as occurring at least 100 days after the transplant.

There is also an overlap syndrome in which people can develop both acute and chronic forms of graft-versus-host disease.


This article will discuss chronic graft-versus-host disease.


Chronic Graft-Versus-Host Disease Symptoms

If you develop chronic graft-versus-host disease after having a bone marrow transplant, you can experience a range of symptoms affecting various areas of the body—such as the skin, mouth, eyes, lungs, and digestive system.2



The condition occurs and produces initial symptoms several months or years after the transplant.3



Symptoms can include any of the following:1


Dry mouth

Mouth sores 

White patches on the mouth 

Dry eyes

A rash

Loss of appetite 

Nausea and vomiting 

Abdominal discomfort 

Diarrhea

Low energy 

Jaundice (yellow discoloration of the skin and eyes)

Shortness of breath 

Predisposition to infections 


You can experience some of these symptoms, but you are unlikely to experience all of them.



Some people have mild effects of chronic graft-versus-host disease, but it can also be very serious. It may cause organ damage, and in some cases, the condition can be life-threatening. 



Causes 

Graft-versus-host disease occurs when the transplanted T-cells attack the transplant recipient’s body. T-cells are a type of white blood cell that play a major role in the immune system. This is seen with allogeneic transplant (receiving another person’s cells), but not after an autologous transplant (receiving your own cells).


Many people who have stem cell transplants and bone marrow transplants do not develop this condition; there are some risk factors that make it more likely. 


Risk factors for graft-versus-host disease include:4


Receiving a transplant from an unrelated or not optimally matched donor 

A history of acute graft-versus-host disease

Advanced age of the transplant recipient 

 What Is My Risk of Graft-Versus-Host Disease?

Diagnosis 

If you have the typical symptoms of graft-versus-host disease, your doctor will examine you and might make a diagnosis based on your symptoms and physical examination.2 Your dentist might notice sores in your mouth, which can be the first sign of the condition.


Sometimes specific diagnostic tests can detect organ involvement. For example, blood tests called liver function tests can indicate the extent of liver damage.


Using National Institutes of Health diagnostic criteria, graft-versus-host disease is scored as mild, moderate, or severe, depending on the severity of organ involvement and the number of organs involved.1


If it isn’t clear whether your symptoms are caused by graft-versus-host disease or another condition, you might need to have diagnostic tests to rule out other issues. 


Conditions that your medical team may need to rule out include: 


Infection: This can cause fevers, discomfort, and a general feeling of being sick. It can be evaluated with a complete blood count (CBC) or culture (sending a sample from the infected area for laboratory evaluation).

Cancer: Cancer can be evaluated with imaging tests or a biopsy (sample of a lump or growth).

Organ rejection: This occurs when the recipient’s immune system rejects the transplant. It can be diagnosed with blood tests.

Treatment 

Several different treatment options are used for graft-versus-host disease.


There is no standard protocol for treatment. Your doctor will prescribe your treatment based on the effects of your condition and whether your symptoms improve with treatment.

This condition may be treated with medication that suppresses the immune system to prevent the donor cells from attacking the recipient cells.


Prednisone, a steroid, is commonly used. It may be started at a low dose and increased gradually, often for approximately a 30-day treatment course. Other immunosuppressant medications, including methotrexate and cyclosporine, have been used as well.


Medications indicated for graft-versus-host disease that did not improve with initial therapy include:


Imbruvica (ibrutinib): This oral medication that comes in tablet and capsule form is a kinase inhibitor indicated for the treatment of adults with chronic graft-versus-host disease after failure of one or more lines of systemic therapy.5

Rezurock (belumosudil): This oral medication that comes in tablet form is a kinase inhibitor indicated for the treatment of adult and pediatric patients 12 years and older with chronic graft-versus-host disease after failure of at least two prior lines of systemic therapy.6

Jakafi (ruxolitinib): This kinase inhibitor oral medication that comes in tablet form is approved for treating chronic graft-versus-host disease after failure of one or two lines of systemic therapy in adult and pediatric patients 12 years and older.7


Kinase inhibitors are medications that interfere with the action of kinase enzymes. These enzymes regulate the activity of specific cells in the body.


The kinase inhibitors used for treating graft-versus-host disease regulate immune cells to prevent them from attacking the cells of the body.


Prognosis 

Graft-versus-host disease may resolve with treatment. Sometimes it can relapse after it has been treated, and it may relapse with different symptoms. The risk of graft-versus-host disease increases with time.


Graft-versus-host disease can cause severe organ damage, and it can be fatal.3 In fact, this condition is the most common fatal complication after a stem cell transplant or a bone marrow transplant. 


Coping 

Living with graft-versus-host disease can be a challenge. It is important to talk to your doctor if you develop symptoms. Treatment can improve the outcome of your condition.


Additionally, you can talk to your medical team about lifestyle modifications and other treatments you can use to ease the discomfort caused by various symptoms, such as dry mouth, nausea, and rash.


 How to Cope If You Have Graft-Versus-Host Disease

Summary

Chronic graft-versus-host disease is a complication of a bone marrow or stem cell transplant. Immune cells (T-cells) from the donor tissue attack the recipient’s cells. To be the chronic form, this occurs 100 days or more after the transplant. It can affect many different areas of the body, including the skin, mouth, eyes, lungs, and digestive system.


The treatment given depends on the effects of the condition and whether they can be controlled. Corticosteroids and other immunosuppressants may be used initially. Kinase inhibitors may be used if there is no improvement.



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