Multidisciplinary Care and Early Patient Engagement in cGVHD

Dr. Shune presents a detailed patient case to illustrate the evaluation and management of moderate cGVHD and the challenges of steroid dependence. The case involves a 52-year-old woman with acute myeloid leukemia who underwent a matched related donor allogeneic stem cell transplant with tacrolimus and methotrexate prophylaxis. She developed grade 2 acute GVHD of the skin and upper gastrointestinal tract at day 30, which initially responded well to systemic corticosteroids. Although she achieved remission at 6 months post-transplant, she experienced recurrence of GVHD at 11 months.

At recurrence, the patient demonstrated classic features of cGVHD, including progressive hyperpigmentation and lichenoid skin changes affecting the forearms and upper back, as well as early sclerotic changes of the lower extremities involving approximately 25% of body surface area. She also had joint involvement with shoulder stiffness limiting daily activities, mild gastrointestinal symptoms without weight loss, and stable pulmonary function testing. Based on NIH consensus criteria, the cumulative organ involvement resulted in a total score of 5, classifying her disease as moderate cGVHD.

Initial systemic therapy with prednisone at 1 mg/kg/day led to only a partial response, prompting steroid tapering due to toxicity concerns. However, the patient experienced repeated disease flares during taper attempts, with worsening skin tightness and increasing body surface area involvement. After persistent disease activity despite multiple tapers, she was categorized as steroid dependent.

Dr. Shune emphasizes that skin manifestations such as hyperpigmentation, lichenoid changes, and sclerosis are highly characteristic of cGVHD. When diagnostic uncertainty exists, particularly in distinguishing GVHD from drug reactions or other dermatologic conditions, dermatology consultation is essential. Skin biopsy is recommended to confirm the diagnosis and exclude alternative etiologies, ensuring appropriate treatment decisions at this advanced stage of disease.