images in haematology
Bilateral optic neuropathy associated with Castleman disease
A 60-year-old woman with a three-day history of decreased vision in her right eye was found to have bilateral optic disc swelling. Visual acuities were 6/9 (right) and 6/6 (left). Colour vision and pupil reactions were normal. There were no signs of uveitis. She had a temporal visual field defect in the right eye but no signs of intracranial space-occupying lesion or venous sinus thrombosis. Cerebrospinal fluid (CSF) opening pressure on lumbar puncture was normal, and CSF was acellular with a protein of 066 g/l. A fundus photograph (top left) and three-dimensional optical coherence scan (bottom left) demonstrated swelling of the right optic disc. Multicentric Castleman disease had been diagnosed 9 years previously on the basis of a right tibial plasmacytoma, lytic lesions in the pelvis and vertebrae, lymphadenopathy and pelvic lymph node biopsy. She was human immunodeficiency virus-negative and had no serum paraprotein or abnormal light chain. Treatment with radiotherapy to the tibia and idarubicin-dexamethasone led to symptomatic and radiological improvement, which persisted until two months before her visual symptoms occurred, when she also developed fatigue and lymphoedema. Imaging demonstrated progression of a lesion in T11, generalized lymphadenopathy, splenomegaly and a left sacral soft tissue mass. A positron emission tomography scan demonstrated intense activity in the left pelvis, and a further plasmacytoma was identified on biopsy. Following her presentation with ocular abnormalities, radiotherapy to the pelvic plasmacytoma and oral prednisolone 50 mg/day were commenced. On tapering prednisolone, the right visual acuity deteriorated to 6/36 with worsening
ª 2014 John Wiley & Sons Ltd, British Journal of Haematology
right optic disc swelling and new right macular oedema being found. Prednisolone was therefore increased to 40 mg/ day followed by a slower taper and rituximab (four cycles) and thalidomide (200 mg/day orally for 6 months) were introduced as steroid-sparing agents. This led to improvement of fatigue and lymphoedema, and resolution of visual symptoms and optic disc and macular oedema, none of which have recurred by 14 months after steroid withdrawal. Fundus photograph (top right) and three-dimensional optical coherence scan (bottom right) demonstrated normal appearances of the right optic disc post-treatment. Ocular involvement in Castleman disease is rare. There is one report of a unilateral optic neuropathy [Kim, U. & Hwang, J.M. (2010) Optic neuropathy associated with Castleman disease. Korean Journal of Ophthalmology, 24, 256– 259]; the patient declined treatment and had a poor visual outcome. Papilloedema (i.e. bilateral optic disc swelling secondary to intracranial hypertension) is a recognized feature of the POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy or oedema, M-protein and Skin abnormalities) that may occur in Castleman disease, but our patient did not have evidence of POEMS. The normal intracranial pressure and response to treatment suggests an inflammatory or infiltrative process of the optic nerve. The macular oedema is likely to be secondary to the optic disc swelling. Simon J. Epps1, Lisa Lowry2 and Catherine M. Guly1 1
Bristol Eye Hospital, Bristol, and 2Bristol Haematology and Oncology
Centre, Bristol, UK. E-mail: [email protected]
doi:10.1111/bjh.13057
Bilateral optic neuropathy associated with Castleman disease
A 60-year-old woman with a three-day history of decreased vision in her right eye was found to have bilateral optic disc swelling. Visual acuities were 6/9 (right) and 6/6 (left). Colour vision and pupil reactions were normal. There were no signs of uveitis. She had a temporal visual field defect in the right eye but no signs of intracranial space-occupying lesion or venous sinus thrombosis. Cerebrospinal fluid (CSF) opening pressure on lumbar puncture was normal, and CSF was acellular with a protein of 066 g/l. A fundus photograph (top left) and three-dimensional optical coherence scan (bottom left) demonstrated swelling of the right optic disc. Multicentric Castleman disease had been diagnosed 9 years previously on the basis of a right tibial plasmacytoma, lytic lesions in the pelvis and vertebrae, lymphadenopathy and pelvic lymph node biopsy. She was human immunodeficiency virus-negative and had no serum paraprotein or abnormal light chain. Treatment with radiotherapy to the tibia and idarubicin-dexamethasone led to symptomatic and radiological improvement, which persisted until two months before her visual symptoms occurred, when she also developed fatigue and lymphoedema. Imaging demonstrated progression of a lesion in T11, generalized lymphadenopathy, splenomegaly and a left sacral soft tissue mass. A positron emission tomography scan demonstrated intense activity in the left pelvis, and a further plasmacytoma was identified on biopsy. Following her presentation with ocular abnormalities, radiotherapy to the pelvic plasmacytoma and oral prednisolone 50 mg/day were commenced. On tapering prednisolone, the right visual acuity deteriorated to 6/36 with worsening
ª 2014 John Wiley & Sons Ltd, British Journal of Haematology
right optic disc swelling and new right macular oedema being found. Prednisolone was therefore increased to 40 mg/ day followed by a slower taper and rituximab (four cycles) and thalidomide (200 mg/day orally for 6 months) were introduced as steroid-sparing agents. This led to improvement of fatigue and lymphoedema, and resolution of visual symptoms and optic disc and macular oedema, none of which have recurred by 14 months after steroid withdrawal. Fundus photograph (top right) and three-dimensional optical coherence scan (bottom right) demonstrated normal appearances of the right optic disc post-treatment. Ocular involvement in Castleman disease is rare. There is one report of a unilateral optic neuropathy [Kim, U. & Hwang, J.M. (2010) Optic neuropathy associated with Castleman disease. Korean Journal of Ophthalmology, 24, 256– 259]; the patient declined treatment and had a poor visual outcome. Papilloedema (i.e. bilateral optic disc swelling secondary to intracranial hypertension) is a recognized feature of the POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy or oedema, M-protein and Skin abnormalities) that may occur in Castleman disease, but our patient did not have evidence of POEMS. The normal intracranial pressure and response to treatment suggests an inflammatory or infiltrative process of the optic nerve. The macular oedema is likely to be secondary to the optic disc swelling. Simon J. Epps1, Lisa Lowry2 and Catherine M. Guly1 1
Bristol Eye Hospital, Bristol, and 2Bristol Haematology and Oncology
Centre, Bristol, UK. E-mail: [email protected]
doi:10.1111/bjh.13057
