Early detection and treatment can help alleviate the severity of the graft-versus-host disease (GVHD)

Among blood cancers and blood disorders we especially point out the graft-versus-host disease (GVHD). 

“It is a complication following the allogeneic stem cell transplantation (SCT)¹. Through this therapy, the patient receives the stem cells of a related or unrelated donor. It is a reaction which is the consequence of immune incompatibility between receiver and donor. The donor’s lymphocytes (a special type of leukocytes, responsible for the immune reaction) recognize the tissues and organs of the patient receiving the donated stem cells as foreign and trigger a rejection reaction on his or her tissue,” 

explains Prof. Irena Preložnik Zupan, PhD, internist and hematologist at the Clinical Department of Hematology of the Internal Clinic at the University Medical Center Ljubljana.

GVHD is the most common cause of death after the allogeneic stem cell transplantation

This potentially fatal complication occurs in 40 to 60% of patients after allogeneic stem cell transplantation (SCT)². In addition to an increased propensity for infection, the graft-versus-host disease is the main medical problem following the allogeneic stem cell transplantation³ and the most common cause of death after the allogeneic stem cell transplantation⁴.

More than 50,000 allogeneic stem cell transplantation procedures are performed each year4, and in the last years, based on the data provided by Prof. Preložnik Zupan, approximately 130 per year are performed in Slovenia, a third of which are allogeneic. To prevent the graft-versus-host disease, patients must be administered immunosuppressants. 

“Patients must be aware of the warning signs of the graft-versus-host disease and immediately call their doctor in case they notice any symptoms and signs. An early detection and treatment can help alleviate the severity of the disease,” 

points out Prof. Preložnik Zupan during the Blood Cancer Awareness Month 2022. 

The signs and symptoms of the graft-versus-host disease

We distinguish the acute and the chronic type of the graft-versus-host disease, known also as the graft-versus-host reaction. Each affects different organs and tissues and has different signs and symptoms. 

Acute GVHD usually develops within the first hundred days after the allogeneic stem cell transplantation. It can affect the skin (rash, blisters or skin flaking off), gastrointestinal tract (nausea, vomiting, abdominal cramps, loss of appetite and diarrhea) and the liver (jaundice).

Chronic GVHD usually develops later. It can affect one or more organs, which is why patients have symptoms on different parts of their bodies: 

• mouth (dry mouth, sensitivity to carbonated drinks or hot, cold, spicy and acidic foods or mint; painful mouth and throat ulcers; difficulty swallowing; gum disease and tooth decay); 
• skin (rashes; dry, tight, itchy skin; thickening and tightening of the skin, which may result in restriction of joint movement; a change in skin color; intolerance to temperature changes due to damaged sweat glands); 
• nails (nail texture change; hard, brittle nails; nail loss); 
• hair and body hair (hair loss, premature graying of the hair); 
• gastrointestinal tract (loss of appetite, unexplained weight loss, nausea, vomiting, diarrhea, abdominal pain); 
• lungs (shortness of breath and difficulty breathing; persistent chronic cough; wheezing); 
• liver (abdominal swelling, jaundice, abnormal liver function test results); 
• muscles and joints (muscle weakness and cramps; joint stiffness which makes it difficult to fully extend the fingers, wrists, elbows, knees, ankles); 
• genitalia (women may experience vaginal dryness, itching and pain; vaginal ulcerations and scarring, narrowing of the vagina, painful intercourse; men may experience narrowing and/or scarring of the urethra; itching and scarring on the penis and scrotum, penis irritation). 

GVHD treatment

The general principles of the graft-versus-host disease treatment include local treatment with glucocorticoids or calcineurin inhibitors in case of a mild disease and systemic treatment in case of a severe disease. 

“The glucocorticoids are the first line of systemic treatment. Second-line medications are added when the patients do not respond to systemic glucocorticoids until the 5th day of treatment or if the symptoms persist after 3 days of treatment. There are many second-line active ingredients, but data on their effectiveness is limited. Often the use of second-line medications is different based on the institution, and there is no consensus among different guidelines on which medication to introduce first. Second-line treatment includes: extracorporeal photopheresis, immunosuppressant treatment, mTOR and JAK inhibitors,” 

explains Prof. Preložnik Zupan.