What Is ECP for GVHD?

Reviewed on 7/9/2020

What Is Graft Versus Host Disease and ECP?

Graft versus host disease (GVHD) is a complication of stem cell and bone marrow transplantation. It is an immune-mediated response that results from an interaction between the donor’s and the recipient’s immunity. It occurs when the donor’s T-cells (the graft) see the patient’s healthy cells (the host) as foreign invaders and launch an immune response to attack them. 

Extracorporeal photopheresis (ECP) is a second-line treatment for GVHD that involves extracting white blood cells, treating them with UV light and medicine, and then re-injecting them into the patient.

Graft-versus-host disease can range from mild to life-threatening.

There are two main forms:

  • Acute GVHD 
    • Develops within the first 100 days following transplantation
    • Can affect the skin, the gastrointestinal tract, or the liver
  • Chronic GVHD
    • Develops after day 100 
    • May involve one or several organs
    • It is a leading cause of medical problems and death after an allogeneic stem cell transplant

What Are Symptoms of Graft Versus Host Disease?

Symptoms of acute graft versus host disease (GVHD) develop within the first 100 days following transplantation and include:

  • Rash 
    • Itchy
    • Painful
    • Burning
    • Red
    • May blister
    • In severe cases, the surface of the skin may flake off
    • May occur on the palms of the hands, soles of the feet, the trunk, and other extremities
  • Liver involvement [yellowing skin and eyes (jaundice), loss of appetite, weight loss, abnormal liver function tests, and rarely, hepatic coma]
  • Gastrointestinal symptoms

Symptoms of chronic graft versus host disease (GVHD) develop within the first 100 days following transplantation and include:

  • Skin
    • Dry, tight, itchy skin
    • Red-to-violet lesions on the palms of the hands, soles of the feet, cheeks, neck, ears, and upper trunk. May progress to involve the whole body.
    • In severe cases, large blisters may form
    • Lichenoid skin lesions or thickening of the skin which may restrict joint movement
    • Intolerance to changes in temperature due to damaged sweat glands
  • Eyes
    • Burning
    • Irritation
    • Sensitivity to light
    • Pain from absence of tears
  • Mouth
    • Mouth dryness
    • Sensitivity to acidic, spicy, hot, or cold foods and carbonated beverages
    • Difficult or painful swallowing
    • Weight loss
    • Painful mouth ulcers inside the cheeks that may extend down the throat
    • Gum disease and tooth decay
  • Lungs
    • Obstructive lung disease
    • Wheezing
    • Shortness of breath
    • Chronic cough 
  • Gastrointestinal tract
  • Liver
    • Abdominal swelling
    • Yellowing skin and eyes (jaundice) from hyperbilirubinemia
    • Abnormal liver function test results
  • Neuromuscular
  • Genitalia 
    • Females
      • Vaginitis 
      • Vaginal dryness, itching, and pain
      • Vaginal ulcerations and scarring
      • Narrowing of the vagina (strictures)
      • Painful sexual intercourse
    • Male 
      • Penile irritation
      • Narrowing and/or scarring of the urethra
      • Itching or scarring on the penis and scrotum
  • Hair and Nails

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What Causes Graft Versus Host Disease?

Graft versus host disease (GVHD) is a complication of cell transplantation caused by an immune-mediated response that results from an interaction between a donor’s and a recipient’s immunity. It occurs when the donor’s T-cells (the graft) see the patient’s healthy cells (the host) as foreign invaders and launch an immune response to attack them.

How Is Graft Versus Host Disease Diagnosed?

Blood tests used to diagnose graft versus host disease (GVHD) include:

Other tests used to diagnose graft versus host disease include:

  • Schirmer test, which measures the degree of tear formation by the tear glands 
  • Pulmonary function tests and arterial blood gas analysis 
  • Manometric studies of the esophagus

Imaging studies used to diagnose graft versus host disease include:

Procedures used to diagnose graft versus host disease include:

What Is the Treatment for Graft Versus Host Disease?

The primary treatment to prevent acute graft versus host disease (GVHD) is: 

  • Cyclosporine for 6 months and 
  • Short-course methotrexate in T-cell–replete allogeneic HCT 
    • Tacrolimus may substitute for cyclosporine because it has a more potent immunosuppressant capacity and lower risk of kidney toxicity
    • Antithymocyte globulin is given before HCT in unrelated-donor transplants

Once a patient has been diagnosed with acute graft versus host disease (GVHD), first-line treatment includes:

If first line treatments for acute graft versus host disease do not work sufficiently, secondary therapy for acute graft versus host disease (GVHD) includes:

  • ATG or multiple pulses of methylprednisolone (at doses higher than those used in initial therapy) 
  • Tacrolimus, for GVHD with cyclosporine resistance or neurotoxicity or nephrotoxicity 
  • Mycophenolate mofetil added to the steroid regimen
  • Infliximab or etanercept 
  • Psoralen and ultraviolet A irradiation (PUVA), for cutaneous lesions 
  • Ruxolitinib 
  • Muromomab-CD3 (Orthoclone OKT3) 
  • Humanized anti-Tac antibody to the IL-2 receptor 

If graft versus host disease (GVHD) becomes chronic, primary therapy includes:

If first line treatments for chronic graft versus host disease do not work sufficiently, secondary therapy for chronic graft versus host disease (GVHD) includes:

  • Ibrutinib for adults who failed at least 1 treatment for chronic GVHD 
  • Mycophenolate mofetil, added to tacrolimus, cyclosporine, sirolimus, and/or prednisone, for steroid-refractory chronic GVHD 
  • Azathioprine, alternating cyclosporine/prednisone, or thalidomide for steroid-refractory chronic GVHD 
  • Clofazimine, for treating skin and oral lesions 
  • Psoralen ultraviolet A (PUVA) therapy, for refractory skin chronic GVHD 
  • Extracorporeal photopheresis (ECP) (a modification of PUVA treatment) in which white blood cells are separated out, then combined with a photoactive drug, exposed to ultraviolet light to “activate” the medicine, and reinfused back into the patient
  • Rituximab, for musculoskeletal and skin chronic GVHD 
  • Pentostatin 
  • Low-dose total lymphoid irradiation to thoracoabdominal areas 
  • Imatinib 

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Reviewed on 7/9/2020
References
Medscape Medical Reference

Leukemia and Lymphoma Society (LLS.org)