Stem cell transplant program

Multidisciplinary program for chronic graft-versus-host disease

Chronic GVHD is a known complication of allogeneic stem cell transplant (when someone receives stem cells from a compatible donor). With chronic GVHD, the new (donor's) immune system attacks the recipient's body. It is similar to an autoimmune process and creates fibrous tissue or scarring. The incidence varies depending upon the stem cell source used, age of patient and donor, and other risk factors. Chronic GVHD may develop following acute GVHD, it may appear after acute GVHD has resolved, or it may occur without any previous acute GVHD. There are many symptoms that need to be evaluated. This chronic condition may affect one or more organs. The chart below contains medical terms you may hear or read about while you or your child is undergoing treatment for chronic GVHD:

Organs affected

Affected Organ	Diagnostic Manifestations	Explanation of Term
Skin
	pruritis	itching
	erythema	red
	macular /papular rash	flat or raised rash, dry scaling skin
	lichen planus-like eruption	pigment or color changes
	poikiloderma	shiny, flat plaques that are red/ blue
	sclerosis	white, hard, movable thickened areas
	deep sclerosis	thick, white tight areas, that may not move
	nail dystrophy	nail ridging, splitting, or loss
	sweat gland loss	not able to sweat, easily flushed with activity
	alopecia	loss of hair
Gastrointestinal (GI) Tract (stomach, esophagus or intestines)
	anorexia	weight loss
	intermittent diarrhea
	abdominal cramping
	esophageal web/ strictures/ stenosis	nausea/ vomiting/ feeling of food being stuck
Liver
	bilirubin greater than 2	lab value indicating liver inflammation
	ALT/ AST increased more than 2x upper limit of normal	lab values indicating liver inflammation
Mouth
	lichen planus-like features	white streaky changes
	plaques
	ulcers
	mucocele	small blisters
	xerostomia	dry mouth
Eye
	dry	less tears, gritty, painful
	photophobia	difficulty with light
	erythema	redness

Genital (vagina)	vaginal scarring and stenosis	narrowing of vagina
	decreased moisture	dry, may be painful or tear
	lichen planus-like changes	unhealthy change in color
Lung
	obstructive lung pattern on pulmonary function test (PFT)	cough, shortness of breath with activity, wheezing
Musculoskeletal (muscles & joints)
	fasciitis	joint stiffness or limited movement
	myositis	severe cramping
Other
	lymphedema (swelling)	poor drainage - intermittent swelling
	serositis	fluid accumulations around the heart and/or lungs

Team approach

Given the complexity of chronic GVHD and the number of organs it can affect, we offer a multidisciplinary program for patients with the condition. The program is open to patients that undergo transplants at the hospital as well as referrals from outside. Patients are seen by the following team members: Morris Kletzel, MD, head of Hematology/Oncology and Stem Cell Transplant; Kimberly Thormann, APN, a nurse-practitioner with expertise in chronic GVHD; an ophthalmologist; a physical therapist; a nutritionist; and a social worker. With this approach, patients have one appointment lasting several hours rather than going to multiple appointments. All health-care practitioners in our program have a specific interest in chronic GVHD. We provide treatment recommendations, both for immunosuppressive therapies as well as for ancillary treatments.

We also ensure that all supportive tests such as pulmonary function tests are occurring at the appropriate intervals. The program facilitates necessary referrals, such as referrals for outpatient therapy. After your child is seen by all the providers, the team meets and decides on a unified set of recommendations, which are then promptly communicated to your child's main transplant physician. We strive to see patients every 3 months during the active phase of their chronic GVHD. Children also have the opportunity to participate in research protocols that deal with finding better ways to monitor and assess chronic GVHD. Children can therefore contribute to helping others, in the future, with chronic GVHD.

Treatment

Standard therapy for chronic GVHD is corticosteroids (prednisone) with a calcineurin inhibitor, such as cyclosporine. Every effort is made to lower prednisone to the lowest amount necessary in order to minimize the side effects of the medication. If your child does not improve on this regimen, we can recommend a number of different therapies. We have significant expertise with extracorporeal photopheresis and pentostatin. More here about extracorporeal photopheresis (ECP) »

Both of these therapies appear to improve sclerotic manifestations of GVHD, which are often very difficult to treat. We also have experience with other medications that can be used to treat GVHD, such as infliximab, etanercept and sirolimus. Some of these recommendations may be made to you/your child depending on the clinical condition and organs affected.