Case 1.
A 19-year-old male with a history of acute myelogenous leukaemia (AML) who underwent reduced intensity allogeneic HSCT (mini-transplantation) in March 2005 was referred to the Keio University Dry Eye outpatient clinic in October 2005 complaining of severe ocular pain, difficulty opening his eyes and foreign body sensations after acute and chronic skin GVHD were completely stabilized. Prior to mini-transplantation, no ocular surface abnormality was noted at the previous hospital, and he started to suffer from newly developed symptoms of DED in May 2005, after alleviation of acute skin GVHD. Systemic prednisolone (PSL) had been tapered from 40 to 20 mg/day in July and was discontinued in August 2005. Just after cessation of PSL, bilateral lid swelling and severe ocular pain had emerged. PSL (15 mg/day) was restarted to treat skin chronic GVHD in October 2005. Skin chronic GVHD was well controlled by this treatment. At the first visit to Keio University Dry Eye outpatient clinic in October 2005, severe DED, extensive trichiasis, corneal conjunctivalization, a corneal ulcer, spontaneous lacrimal punctal occlusion, and active corneal neovascularization were observed in both eyes (Fig. 1). Best-corrected visual acuity (BCVA) was 20/100 in his right eye and 20/32 in his left eye. Although the patient had been treated with all the available conventional pharmacotherapies, ocular inflammation was not sufficiently controlled. Therefore, we decided to perform AMT on both eyes in October 2005. After releasing the symblephara, the AM was implanted not only onto the surface of the bulbar conjunctiva but also onto the deep fornices. Three months after AMT, the fornices were deep and well maintained, and after 9 months, visual acuity improved to 20/25 in his right eye and 20/22 in his left eye. Contact lens was used to alleviate severe ocular pain due to extensive trichiasis, but a corneal perforation of unknown aetiology on his right eye occurred one year after AMT. Penetrating keratoplasty was performed on his right eye without complications, and no rejection has occurred for more than 12 years. Systemic GVHD progression has also not been observed during this time.
Case 2.
A 59-year-old female with a history of mixed phenotype acute leukaemia (lymphoid 60% and myeloid 40%) who underwent allogeneic bone marrow transplantation (BMT) without irradiation in May 2010 was referred to the Keio University Dry Eye outpatient clinic in September 2013 as she developed refractory chronic oGVHD.
After BMT, she developed acute skin GVHD and was successfully treated with systemic tacrolimus (2 mg/day) and PSL (30 mg/day), which was tapered over almost one year. In early 2011, just after tapering of treatment for acute skin GVHD, she developed DED and transient difficulty opening her mouth, which was diagnosed as chronic ocular and oral GVHD. She had been suffering from symblepharon, LSCD and conjunctivalization in her right eye, and she had been previously treated with contact lenses, commercially available eye drops, autologous serum and topical cyclosporine and tacrolimus, yet her ocular surface condition had not improved. At the first visit to Keio University Hospital in 2013, severe conjunctival fibrosis of the upper conjunctiva and fornix shortening, partial LSCD and conjunctivalization of the upper cornea on her right eye were observed (Fig 2) despite no sign of active systemic GVHD. BCVA was 20/100 in her right eye and 20/20 in her left eye. Therefore, we decided to perform AMT on the right eye. Conjunctival fibrotic tissue was removed from the upper fornix to the limbus, and AMT was performed as a substrate and to cover the ocular surface. The patient was able to undergo cataract surgery on the right eye 1 year after AMT as improvement of corneal clarity in the pupillary area of the right eye was achieved. Two years after AMT, there was only a minor recurrence of corneal conjunctivalization, and her visual acuity was 20/28 in her right eye. She reported that her daily life activity had been satisfactory without any inconvenience for more than 27 months since AMT. However, corneal conjunctivalization of the right eye progressed slowly, and AMT with allogenic cultivated limbal epithelial transplant was performed on the right eye. Her postoperative course has been good for more than 5 months after this procedure. Systemic GVHD progression has not been observed postoperatively.