title: Sclerodermatous graft-versus-host disease: clinical spectrum and therapeutic challenges.
abstract: Sclerodermatous graft-versus-host disease (GVHD) is a rare complication of bone marrow transplantation. While GVHD is often associated with the beneficial graft vs. tumour effect, it also contributes towards significant morbidity and mortality. No reliably effective treatment has yet been established. We present 10 patients with haematological malignancies who underwent an allogeneic stem cell transplant and developed sclerodermatous GVHD. Donor lymphocyte infusion administered for relapse or reducing donor T-cell chimerism was a known trigger for sclerodermatous GVHD in four of the patients. Treatment with immunosuppressants, psoralen plus ultraviolet A (PUVA) and extracorporeal photopheresis has been largely unsuccessful in their management. Intensive immunosuppression including the use of anti-CD20 monoclonal antibody may have contributed to relapse of leukaemia in one patient 10 years after her transplant. Sclerodermatous GVHD may occur without a preceding lichenoid stage. Clinical heterogeneity is common, although sclerodermatous GVHD has a predilection for the limbs. Treatment options are largely unsatisfactory if conventional immunosuppression fails. PUVA may give some symptomatic benefit and extracorporeal photopheresis seems to be less efficacious than previously published work suggests.
authors.affiliation: Department of Dermatology, King's College Hospital, London, UK. jonathanmlwhite@hotmail.com
authors.forename: J M L
authors.initials: JM
authors.lastname: White
authors.forename: D
authors.initials: D
authors.lastname: Creamer
authors.forename: A W P
authors.initials: AW
authors.lastname: du Vivier
authors.forename: A
authors.initials: A
authors.lastname: Pagliuca
authors.forename: A Y
authors.initials: AY
authors.lastname: Ho
authors.forename: S
authors.initials: S
authors.lastname: Devereux
authors.forename: J R
authors.initials: JR
authors.lastname: Salisbury
authors.forename: G J
authors.initials: GJ
authors.lastname: Mufti
mesh_terms: D000328:Adult; D000368:Aged; D000753:Anemia, Refractory / Q000601:surgery; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D005260:Female; D006689:Hodgkin Disease / Q000601:surgery; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007951:Leukemia, Myeloid / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011701:PUVA Therapy; D010954:Plasmacytoma / Q000601:surgery; D012008:Recurrence; D012594:Scleroderma, Localized / Q000145:classification / Q000209:etiology / Q000628:therapy*; D033581:Stem Cell Transplantation / Q000009:adverse effects; D013922:Thrombocytosis / Q000601:surgery; D017211:Treatment Failure
chemical_list: D007166:Immunosuppressive Agents