Leukemia Research Vol. 16. No. 4, pp. 411413, 1992. Printed in Great Britain.

0145-2126/92$5.00 + .00 Pergamon Press pie

CASE REPORT ISOLATED OPTIC NERVE INVOLVEMENT IN CHRONIC MYELOID LEUKEMIA CIRO COSTAGLIOLA, MARIO RINALDI, LUIGI COTTICELLI, SANDRO SBORDONE and GIACOMO NASTRI

Eye Clinic, 1st School of Medicine, University of Naples, Naples, Italy

(Received 2 July 1991. Accepted 30 August 1991) Abstract--Eye involvement in chronic myeloid leukemia is well known. The signs are infiltration of the choroid, the sclera, the episclera, the conjunctiva and the optic nerve. Eye involvement is more common in acute than in chronic forms of leukemia. This paper reports a case of chronic myeloid leukemia in which optic nerve involvement was the only initial sign of the disease.

Key words: Chronic myeloid leukemia, optic nerve, computerized visual field, electroretinogram, visual evoked potentials.

normal; fluorescein angiography, normal; computerized visual field, normal. Left eye. Visual acuity, 20/20; IOP, 16.5 mmHg; fundus, at the upper-nasal edge of the optic disk a slight oedema and a modest hyperemia were found. There was also a marked vascular tortuosity, particularly at the site of the infiltration. Fluorescein angiography showed a slight hyperfluorescence of the optic nerve head; no leakage and/or vessel wall alterations occurred. Choroid normal. Computerized visual field: the blind spot appeared slightly increased; two very small peripheral defects were present. We also conducted electroretinographic (ERG) and visual evoked potentials (VEP) examinations. While ERG and pattern ERG (PERG) were normal in both eyes, the pattern VEP of the left eye showed a delayed latency at low frequencies. The flash-VEP showed no significant differences either in latency or in amplitude in both eyes, as compared to our normal laboratory standard. Hemogram. Erythrocytes 3 810 000/mm 3 (light anysoand poikilo-cytosis); leukocytes 34 800/mm 3 (with presence of immature forms); platelets 580 000/ram 3. Routine blood tests. Negative.

INTRODUCTION EVE involvement in leukemia is well known [1]. Ocular manifestations are sometimes so typical that they may form the basis for diagnosis [2]. However, ophthalmological findings are m o r e c o m m o n in the acute than in chronic forms [3]. Infiltration is most often observed at the choroidal level [4]; also the sclera, episclera, conjunctiva, retina and optic nerve show typical infiltrations [5]. The optic nerve might present neuritis with consequent atrophy, papilledema or, m o r e rarely, neovascularization [6, 7]. Isolated optic nerve involvement is uncommon. Previous authors have described cases of isolated optic nerve neuropathy in leukemia [8, 9, 10], but these were always seen in acute leukemia with the exception of the case reported by Ellis et al. [9] in which the leukemia was chronic. M o r e recently, Kinkaid and G r e e n [11] described a possibly isolated optic nerve infiltration in a chronic form of leukemia. H e r e we report a case of chronic myeloid leukemia in which optic nerve involvement was the only initial sign of the disease.

Leukocyte alkaline phosphatase cytochemical reaction: negative.

General medical examination: negative. Neurological examination: negative. Cranial and orbit computerized axial tomography: negative. Cerebrospinal fluid examination: negative. The peripheral blood picture was confirmed by a bone marrow biopsy. At this point a chronic myeloid leukemia was diagnosed.

CASE REPORT A 53-year-old presented in our clinic for a routine ocular examination. He had no symptoms of ocular disease.

Clinic examination Right eye. Visual acuity, 20/20; IOP, 17 mmHg; fundus,

DISCUSSION

Correspondence to: Dr C. Costagliola, M.D., Via F. Petrarca 41/A, 80122 Napoli, Italy.

Optic nerve involvement was the only s y m p t o m of the disease in our patient. The o e d e m a t o u s and 411

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slightly hyperemic appearance of the optic disk suggested the presence of a systemic disease. The optic disk is often affected in many systemic diseases, such as diabetes, arterial hypertension, polycytemia, but usually at a late stage, when all the general findings are already evident. The case reported shows that the optic nerve can be involved by pathological processes earlier and to a greater extent than other ocular tissues. It is difficult to ascertain whether the optic nerve involvement is due to a specific leukemic infiltrate, to vessel wall alteration or to a disorder in the blood flow rate. However, our patient showed a very great increase of the platelets and this could be responsible for vessel micro-thrombosis on the optic disk. This is supported by the finding that chronic myeloid leukemia is often associated to an increase in the platelets [18]. This effect could also explain the relatively high frequency of the central retina vein thrombosis in this disease. The electrophysiological results also confirm the presence of an altered nervous conduction due probably to a reduced blood flow rate at the level of the optic disk vessels. In fact, the normality of E R G , P E R G and flash-VEP indicates both retinal integrity and normal function of ganglion cells; the delayed latency of P 1(/0 in the pattern VEP assigns the alteration at the optic nerve fiber level. Moreover, the finding of the defect only at low frequencies demonstrates the involvement of the more peripheral optic nerve fibers and could also explain the normal visual acuity and the visual field results [13, 14]. Culler [15] reported that a leukemic-induced optic disk oedema is not always closely related to an obvious increase of intra-cranial pressure; leukemic infiltration of both retinal central vessels and the vascular tree of the perioptical pial septa is able to produce a venous congestion a n d / o r an ischemia with relative oedema [16]. In conclusion, leukemia-induced optic disk oedema could be attributed to the following causes: (1) central nervous system involvement with a consequent increase of intra-cranial pressure; (2) orbital pseudotumoral leukemic infiltration; (3) venous congestion due to perivascular leukemic infiltration; (4) optic disk localized leukemic infiltration; (5) diminished blood flow rate due to an increase in platelets and leukocytes. As early as 1986, Stephens [17] observed that when the number of leukocytes is equal to or above 5 0 0 0 0 / m m 3 blood hyperviscosity occurs.

The last mechanism is in accordance with the ophthalmological picture in our patient. Acknowledgements--Thanks are due to Mr A. Giacoia for his excellent technical assistance.

REFERENCES 1. Krebs W., Metz O. & Plenert W. (1982) Okulare Syntomatik und Leukemie im Kindesalter. Klin. Mbl. Augenheilk 181,407-408. 2. Martin-Bousset D., Lawki F., Baudu P. & Moreau P. (1981) Syndrome d'ischemie aigue choriocapillaire au cours d'une leucose. Reflexion/a-propos d'une observation. J. Fr. Ophthalmol. 5, 609-613. 3. Chumbley L. C. (1981) Hematologic-oncologic disorders. Ophthalmological Interne Medicine, p. 193. Saunders Company, Philadelphia. 4. Miglior M., Masora G., Lasagni F. & Lambertenghi E. (1987) La uveopatia leucemica: contributo clinico. Boll. Ocul. 63, 467-473. 5. Rosenthal A., Egbert P., Wilbur J. & Probert J. (1975) Leukemic involvement of the optic nerve. J. Pediat. Ophthalmol. 12, 84-87. 6. De Juan E., Green R., Rice T. & Erozan Y. (1982) Optic disk neovascularization associated with ocular involvement in acute lymphocytic leukemia. Retina 2, 61-64. 7. Delaney W. V. & Kinsella G. (1985) Optic disk neovascularization in leukemia. Am. J. Ophthalmol. 99, 212-213. 8. A1-Rashid R. (1971) Papilloedema and ataxia as initial manifestation of acute lymphocytic leukemia. J. Pediat. Ophthalmol. 8, 29-30. 9. Ellis W., Hunter L. & Little M. (1973) Leukemic infiltration of the optic nerve head. Am. J. Ophthalmol. 75, 867-871. 10. Nitschke R., Balyeat H. & Taylor T. (1981) Leukemic optic nerve infiltration 17 months after cessation of therapy. Am. J. Pediat. Hemat. Oncol. 3, 17-19. 11. Kinkaid M. & Green R. (1983) Ocular and orbital involvement in leukemia survey. Ophthalmol. 27, 4-8. 12. Ballantyne A. & Michaelson I. (1962) The Fundus of the Eye, p. 220. Williams and Wulkens. 13. Porciatti W. &von Berger G. P. (1981) Visual potential evoked by pattern stimulation with different spatial frequencies in retrobulbar neuritis. Doc. Ophthalmol. Proc. Ser. 27, 67-75. 14. Porciatti V. (1985) Electrophysiological diagnosis in optic nerve disease by combined use of pattern electroretinograms and visual evoked potentials. In Evoked Potentials Neurophysiological and Clinical Aspects (Morocutti C. & Rizzo P. A., Eds), p. 281. Elsevier, Holland. 15. Culler A. M. (1951) Fundus change in leukemia. Trans. Am. Ophthalmol. Soc. 49, 445--473. 16. Takahashi T., Oda Y. & Isayama Y. (1982) Leukemic optic neuropathy. Ophthalmologica 185, 37-45. 17. Stephens D. (1936) Relation of viscosity of blood to leucocyte count with particular reference to chronic myelogenous leukemia. Proc. Soc. Expl Biol. Med. 35, 251-260.

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Isolated optic nerve involvement in chronic myeloid leukemia.

Eye involvement in chronic myeloid leukemia is well known. The signs are infiltration of the choroid, the sclera, the episclera, the conjunctiva and t...
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