Clinical features:
(1) The patient has a history of allogeneic HSCT.
(2) The patient develops fibrosis of the skin and/or fascia.
(3) Other causes of sclerotic skin are excluded.
Risk factors may include:
(1) total body irradiation, especially with reduced-intensity conditioning
(2) genetic risk factors
The patient may have certain genetic risk factors:
(1) presence of CD247 GG or GT genotype in donor or recipient
(2) presence of rs987870 CC genotype (5'-flanking region of HLA-DPA1) in donor or recipient
(3) absence of BANK1 TT genotype in donor (presence associated with reduced risk)
Complications may include:
(1) skin breakdown and poor wound healing
(2) joint contractures
(3) restricted chest expansion
Extensive sclerosis is associated with poor quality of life, increased disability and poor survival.
