title: Retinal Involvement in a Patient with Cerebral Manifestation of Chronic Graft-Versus-Host-Disease.
abstract: BACKGROUND
  We report a 35-year-old female patient with cerebral manifestations of chronic graft-versus-host disease (cGvHD) and putative retinal involvement after allogeneic peripheral blood stem cell transplantation (alloHSCT).
  
  
  METHODS
  The patient experienced recurrent episodes of fever and encephalitic signs 7 months after alloHSCT during taper of immunosuppression (IS).
  
  
  RESULTS
  Cerebral magnetic resonance imaging (MRI) showed non-gadolinium-enhancing confluent periventricular lesions and cerebrospinal fluid inflammation. After exclusion of infectious causes, treatment with steroids and antiepileptics improved cognitive deficits. Steroid reduction provoked a relapse responding to IS. 2 years later, she complained of right-sided blurred vision and floaters; both eyes showed whitish circumscribed retinal infiltrations, cellular infiltration of the vitreous and mild bilateral optic disc edema. Oncological and neurological work-up ruled out infectious diseases and other GvHD manifestations. Symptoms and signs resolved under continued systemic IS, leaving pigmented retinal scars. After IS withdrawal, classical cutaneous cGvHD developed, resolving on systemic IS. 94 months after transplantation, she is doing well.
  
  
  CONCLUSIONS
  To our knowledge, this is the first observation of retinal involvement of cerebral cGvHD manifestations with retinal infiltrations documented in the absence of other causes and in parallel to periventricular lesions in cerebral MRI. Based on bone marrow histology, we discuss a small vessel pathophysiology of cGvHD.
authors.affiliation: Department of Ophthalmology, University Medical Center Regensburg, Germany.
authors.forename: Christiane
authors.initials: C
authors.lastname: Blecha
authors.forename: Klemens
authors.initials: K
authors.lastname: Angstwurm
authors.forename: Daniel
authors.initials: D
authors.lastname: Wolff
authors.forename: Ernst
authors.initials: E
authors.lastname: Holler
authors.forename: Horst
authors.initials: H
authors.lastname: Helbig
authors.forename: Jochen
authors.initials: J
authors.lastname: Grassinger
authors.forename: Janine
authors.initials: J
authors.lastname: Rennert
authors.forename: Ingo
authors.initials: I
authors.lastname: Kleiter
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Huber
authors.forename: Christoph
authors.initials: C
authors.lastname: Schmid
authors.forename: Tina
authors.initials: T
authors.lastname: Dietrich-Ntoukas
mesh_terms: D000328:Adult; D002908:Chronic Disease; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans; D045164:Mesenchymal Stem Cell Transplantation / Q000009:adverse effects*; D012164:Retinal Diseases / Q000175:diagnosis / Q000209:etiology*