Clin. lab. Haemat. 1991, 13, 217-220
ADONIS 014198549100027 2
CASE REPORT
Retinal detachments due to chronic lymphocytic leukaemia J.A. M U R P H Y , M R C P * , J . F . PITTS, MRCPT, J . D U D G E O N , F R C S t , R.B. H O G G , F R C P * & A.N. HARNETT, FRCRS *Department of Clinical and Laboratory Haematology, Stobhill General Hospital, Glasgow G21 3UW, TTennent Institute of Ophthalmology, Western Injirmary, Glasgow GI I 6 N T and $Beatson Oncology Centre and Tennent Institute of Ophthalmology, Western Infirmary, Glasgow Accepted for publication 2 March 1990 Keywords: retinal detachment, leukaemia, choroid, orbital radiotherapy
Ocular involvement in acute leukaemia is well-recognized; it presents clinically in less than 10% but at postmortem in over 50% of patients (Ridgway et al. 1976; Kincaid & Green 1983). However, in chronic lymphocytic leukaemia (CLL), limited ocular involvement has not been reported. We report a patient with CLL in whom visual disturbance was shown to be due to choroidal infiltration which was treated with local radiotherapy. Case report
A 6 1 -year-old man with bulky superficial and extensive retroperitoneal lymphadenopathy and splenomegaly was diagnosed as having CLL due to a high lymphocyte count (635 x 109/1), infiltration of bone marrow by moderately well differentiated lymphocytes and repeated immunophenotyping which was typical of B cell CLL. He was anaemic (haemoglobin 7.1 g/dl) and thrombocytopenic (platelet count 23 x 109/1) (with no evidence that these were immune in origin) and, therefore, classified as Stage IV (Rai) or Stage C (Binet). Soon after admission the patient developed nodules in both thighs. Biopsy revealed extensive infiltration by small, mature lymphocytic cells, similar to those seen in the bone marrow. Correspondence: Dr J. A. Murphy, Department of Haematology, Glasgow Royal Infirmary, Glasgow G4 OSF.
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The patient was commenced on oral Prednisolone and Chlorambucil. The nodules over the thighs regressed. Three months after presentation there had been virtually complete resolution of superficial lymphadenopathy, and the spleen had reduced in size. His circulating lymphocyte count had fallen to 420 x 109/l.The anaemia and thrombocytopenia had improved. Five months after presentation, and while remaining on Chlorambucil and Prednisolone, he complained of deteriorating vision and he was referred for ophthalmological assessment. Examination revealed visual acuity in the right eye was hand movements appreciated in the temporal field. In the left eye visual acuity was 2/60. Apart from early lens changes which were compatible with his age the anterior segments were unremarkable. The vitreous gel was packed with white cells in both eyes. The optic discs appeared normal. There were bilateral shallow retinal detachments with aggregates of white fluffy material in the subretinal space in both eyes, and in the right eye there was one large collection of this material on the retinal surface (Figure 1). We considered the need for a tissue diagnosis in the form of vitreous aspiration or choroidal biopsy but in view of the patient’s debility and the significant risks of these procedures this was not pursued. The ocular findings were those of a bilateral diffuse infiltrative process and taken with the haematological findings a clinical diagnosis of choroidal involvement by CLL was made.
Figure 1. Left fundus showing retinal detachment (upper two-thirds of photograph), retinal haemorrhages, and sub-retinal leukaemic infiltrates (arrowed).
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Neurological examination was normal apart from the ocular findings described and examination of the cerebro-spinal fluid was normal. He was treated with a course of bilateral orbital radiotherapy using megavoltage radiation. Anterior fields were employed and he received 24 Gy in 12 fractions over 16 days. This treatment was well tolerated. Within seven days of starting radiotherapy the cellular infiltrate in the vitreous gel had cleared in both eyes. At three months the infiltrates in the left eye had disappeared and the detachments had resolved. The vision in the left eye did not improve, however, due to retinal traction in the macular area and the acuity remained 2/60. The disc remained normal. In the right eye the infiltrates disappeared but the detachments persisted and the visual acuity remained at hand movements. Twenty months after presentation his vision has remained static, he has minimal superficial lymphadenopathy but splenomegaly persists and his haematological parameters are haemoglobin 1I .O g/dl, lymphocyte count 30 x 109/1, and platelets 30 x 109/l. He continues on Chlorambucil 2 mg and Prednisolone 10 mg daily. He is coping independently at home. Discussion Choroidal infiltration is recognized as one of the forms of ocular involvement in acute leukaemias and has been reported in chronic granulocytic leukaemia, as well as in malignant lymphocytic lymphomas (Rosenthal 1983; Weisenthal et al. 1988). In only one previous report has choroidal involvement been recognized clinically in CLL and in this it was accompanied by extensive involvement elsewhere in the eye (Newman et al. 1972). This is the first report of CLL clinically limited to the posterior segment of the eye. As well as the choroidal infiltration which developed while on chemotherapy, this patient represents an unusual case of CLL because of the very high lymphocyte count at presentation, the extra-nodal (skin) involvement, and survival beyond twelve months despite advanced stage at presentation. We believe that clinicians should be aware of the possibility of direct ocular involvement in CLL. Localized treatment with radiotherapy is worthy of consideration especially as response may be obtained from low doses which are unlikely to be attended by complications. Acknowledgement We thank Dr N. P. Lucie for the cell marker studies. References KINCAIDM.C. & GREENW.R. (1983) Ocular and orbital involvement in leukaemia. Surv. Ophthahol. 27, 21 1-232
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NEWMAN N.M., SMITH M.E. & GAYA.J. (1972) An unusual case of leukaemia involving the eye: a clinico-pathological study. Surv. Ophthalmol. 16, 3 16-321 RIDCWAY E.W., JAFFE N. & WALTOND.S. (1976) Leukaemic ophthalmopathy in children. Cancer 38, 1744-1749 ROSENTHAL A.R. (1983) Ocular manifestations of leukaemia. Ophthalmology 90, 899-905 WEISENTHAL R., FRAYER W.C., NICHILS C.W. & EAGLER.C. (1988) Bilateral ocular disease as the initial presentation of malignant lymphoma. Br. J. Ophthalmol. 72, 248-252
ADONIS 014198549100027 2
CASE REPORT
Retinal detachments due to chronic lymphocytic leukaemia J.A. M U R P H Y , M R C P * , J . F . PITTS, MRCPT, J . D U D G E O N , F R C S t , R.B. H O G G , F R C P * & A.N. HARNETT, FRCRS *Department of Clinical and Laboratory Haematology, Stobhill General Hospital, Glasgow G21 3UW, TTennent Institute of Ophthalmology, Western Injirmary, Glasgow GI I 6 N T and $Beatson Oncology Centre and Tennent Institute of Ophthalmology, Western Infirmary, Glasgow Accepted for publication 2 March 1990 Keywords: retinal detachment, leukaemia, choroid, orbital radiotherapy
Ocular involvement in acute leukaemia is well-recognized; it presents clinically in less than 10% but at postmortem in over 50% of patients (Ridgway et al. 1976; Kincaid & Green 1983). However, in chronic lymphocytic leukaemia (CLL), limited ocular involvement has not been reported. We report a patient with CLL in whom visual disturbance was shown to be due to choroidal infiltration which was treated with local radiotherapy. Case report
A 6 1 -year-old man with bulky superficial and extensive retroperitoneal lymphadenopathy and splenomegaly was diagnosed as having CLL due to a high lymphocyte count (635 x 109/1), infiltration of bone marrow by moderately well differentiated lymphocytes and repeated immunophenotyping which was typical of B cell CLL. He was anaemic (haemoglobin 7.1 g/dl) and thrombocytopenic (platelet count 23 x 109/1) (with no evidence that these were immune in origin) and, therefore, classified as Stage IV (Rai) or Stage C (Binet). Soon after admission the patient developed nodules in both thighs. Biopsy revealed extensive infiltration by small, mature lymphocytic cells, similar to those seen in the bone marrow. Correspondence: Dr J. A. Murphy, Department of Haematology, Glasgow Royal Infirmary, Glasgow G4 OSF.
217
21 8
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The patient was commenced on oral Prednisolone and Chlorambucil. The nodules over the thighs regressed. Three months after presentation there had been virtually complete resolution of superficial lymphadenopathy, and the spleen had reduced in size. His circulating lymphocyte count had fallen to 420 x 109/l.The anaemia and thrombocytopenia had improved. Five months after presentation, and while remaining on Chlorambucil and Prednisolone, he complained of deteriorating vision and he was referred for ophthalmological assessment. Examination revealed visual acuity in the right eye was hand movements appreciated in the temporal field. In the left eye visual acuity was 2/60. Apart from early lens changes which were compatible with his age the anterior segments were unremarkable. The vitreous gel was packed with white cells in both eyes. The optic discs appeared normal. There were bilateral shallow retinal detachments with aggregates of white fluffy material in the subretinal space in both eyes, and in the right eye there was one large collection of this material on the retinal surface (Figure 1). We considered the need for a tissue diagnosis in the form of vitreous aspiration or choroidal biopsy but in view of the patient’s debility and the significant risks of these procedures this was not pursued. The ocular findings were those of a bilateral diffuse infiltrative process and taken with the haematological findings a clinical diagnosis of choroidal involvement by CLL was made.
Figure 1. Left fundus showing retinal detachment (upper two-thirds of photograph), retinal haemorrhages, and sub-retinal leukaemic infiltrates (arrowed).
Retinal detachments due to CLL
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Neurological examination was normal apart from the ocular findings described and examination of the cerebro-spinal fluid was normal. He was treated with a course of bilateral orbital radiotherapy using megavoltage radiation. Anterior fields were employed and he received 24 Gy in 12 fractions over 16 days. This treatment was well tolerated. Within seven days of starting radiotherapy the cellular infiltrate in the vitreous gel had cleared in both eyes. At three months the infiltrates in the left eye had disappeared and the detachments had resolved. The vision in the left eye did not improve, however, due to retinal traction in the macular area and the acuity remained 2/60. The disc remained normal. In the right eye the infiltrates disappeared but the detachments persisted and the visual acuity remained at hand movements. Twenty months after presentation his vision has remained static, he has minimal superficial lymphadenopathy but splenomegaly persists and his haematological parameters are haemoglobin 1I .O g/dl, lymphocyte count 30 x 109/1, and platelets 30 x 109/l. He continues on Chlorambucil 2 mg and Prednisolone 10 mg daily. He is coping independently at home. Discussion Choroidal infiltration is recognized as one of the forms of ocular involvement in acute leukaemias and has been reported in chronic granulocytic leukaemia, as well as in malignant lymphocytic lymphomas (Rosenthal 1983; Weisenthal et al. 1988). In only one previous report has choroidal involvement been recognized clinically in CLL and in this it was accompanied by extensive involvement elsewhere in the eye (Newman et al. 1972). This is the first report of CLL clinically limited to the posterior segment of the eye. As well as the choroidal infiltration which developed while on chemotherapy, this patient represents an unusual case of CLL because of the very high lymphocyte count at presentation, the extra-nodal (skin) involvement, and survival beyond twelve months despite advanced stage at presentation. We believe that clinicians should be aware of the possibility of direct ocular involvement in CLL. Localized treatment with radiotherapy is worthy of consideration especially as response may be obtained from low doses which are unlikely to be attended by complications. Acknowledgement We thank Dr N. P. Lucie for the cell marker studies. References KINCAIDM.C. & GREENW.R. (1983) Ocular and orbital involvement in leukaemia. Surv. Ophthahol. 27, 21 1-232
220
J . A . Murphy et al.
NEWMAN N.M., SMITH M.E. & GAYA.J. (1972) An unusual case of leukaemia involving the eye: a clinico-pathological study. Surv. Ophthalmol. 16, 3 16-321 RIDCWAY E.W., JAFFE N. & WALTOND.S. (1976) Leukaemic ophthalmopathy in children. Cancer 38, 1744-1749 ROSENTHAL A.R. (1983) Ocular manifestations of leukaemia. Ophthalmology 90, 899-905 WEISENTHAL R., FRAYER W.C., NICHILS C.W. & EAGLER.C. (1988) Bilateral ocular disease as the initial presentation of malignant lymphoma. Br. J. Ophthalmol. 72, 248-252
