Clinical aspects of sclerodermatous type graft-versus-host disease after allogeneic hematopoietic cell transplantation Allogeneik hematopoietik hücre nakli sonrası sklerodermatoz tip graft-versus-host hastalıǧının klinik yönleri


ŞANLI H., AKAY B. N., SOYDAN E., KOÇYİĞİT P., Arat M., İLHAN .

Turkish Journal of Hematology, cilt.27, sa.2, ss.91-98, 2010 (Scopus) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 27 Sayı: 2
  • Basım Tarihi: 2010
  • Doi Numarası: 10.5152/tjh.2010.06
  • Dergi Adı: Turkish Journal of Hematology
  • Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.91-98
  • Anahtar Kelimeler: Allogeneic hematopoietic stem cell transplantation, Sclerodermatous graft-versus-host disease
  • Lokman Hekim Üniversitesi Adresli: Evet

Özet

Objective: We aimed to evaluate the clinical features of sclerodermatous chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (AHSCT). Materials and Methods: We retrospectively analyzed 423 patients who underwent AHSCT. We assessed age, sex, pre-transplant diagnosis, conditioning regimen, GVHD prophylaxis, and occurrence of acute GVHD (aGVHD), chronic lichenoid and chronic systemic GVHD, and clinical properties of sclerodermatous GVHD. Results: Sclerotic skin lesions developed in 22 patients after a mean of 752±647 days (median 480). aGVHD appeared in 17 patients, with hepatic involvement in 2, gastrointestinal tract involvement in 2 and skin involvement in 13 of these patients. Extensive chronic GVHD (liver, pulmonary, skin and oral mucosa) developed in 12 patients. Sclerosis was generalized in 19 patients (86.4%) and localized in 3 patients (13.6%). Leopard skin eruption appeared in 8 (36.4%) of the 19 patients with generalized sclerodermatous changes. In most cases, sclerotic lesions appeared on the trunk, and distal parts of the extremities were spared. Eight patients (36.4%) progressed from lichenoid to sclerodermatous lesions, 2 (9.1%) with lichenoid and sclerodermatous phases together and 12 (55.5%) with de novo sclerodermatous lesions. Five patients died because of late transplant-related complications. Conclusion: Sclerodermatous GVHD has a late onset and may be quite disabling. Unlike scleroderma, acral involvement is seen rarely. Although most lesions do not disappear in the course of the disease, most patients have a good prognosis.