SEROUS RETINAL DETACHMENT AS A PRESENTING FEATURE OF CHRONIC MYELOGENOUS LEUKEMIA Javier Montero, MD, Enrique Cervera, PhD, Paula Palomares, MD, Luis Amselem, MD, Manuel Diaz-Llopis, PhD
Purpose: The purpose of this study was to report a case of diffuse unilateral retinal detachment as an initial symptom of chronic myelogenous leukemia in a 35-year-old woman. Methods: This is a case report of a 35-year-old white woman who presented with painless acute visual loss in her left eye. Results: A 35-year-old white woman presented with acute visual loss in her left eye. She had no systemic or ophthalmological medical history. Slit-lamp examination was normal in both eyes. Fundus examination of the left eye showed not only multiple retinal hemorrhages but also a diffuse serous retinal detachment affecting the nasal aspect of the retina and the posterior pole. The leukocyte count was 124,000/mm3 (99% blasts), and the Philadelphia chromosome was positive, an abnormality that is associated with chronic myelogenous leukemia. Conclusion: Serous retinal detachment is an uncommon feature among leukemias. Chronic myelogenous leukemia may remain asymptomatic until a blastic phase occurs. Prompt recognition of the disease leads to early systemic chemotherapy and may help to restore visual function. RETINAL CASES & BRIEF REPORTS 4:394–396, 2010
rescence corresponding to window defects and blockage caused by retinal pigment epithelium atrophy and clumping with one or more areas of subtle continued leakage. During the later phases of the angiogram, these areas gradually enlarged and stained the adjacent subretinal and subretinal pigment epithelium fluid (Figure IB). Optical coherence tomography confirmed a serous RD with a great amount of fluid, measuring .1,000 mm (Figure 1C). A complete laboratory examination, including complete blood count, autoantibody (antineutrophil cytoplasmic antibodies [ANCA], antinuclear antibodies [ANA]), venereal disease research laboratory search, erythrocyte sedimentation rate, C-reactive protein, and Mantoux test, was performed. In addition, a chest x-ray, an ocular ultrasound, and a chest examination were performed. The chest x-ray and the chest examination showed no pathologic findings. The ocular ultrasound showed an RD with a subtle choroidal thickening (Figure ID). The Mantoux test was negative.
From the Department of Ophthalmology, Hospital General Universitario de Valencia, Valencia, Spain.
A
35-year-old white woman presented with painless acute visual loss in her left eye. Her bestcorrected visual acuity was 20/20 in the right eye and 20/40 in the left eye. She had no systemic or ophthalmological medical history. Slit-lamp examination was normal. Fundus examination of the left eye showed not only multiple retinal hemorrhages but also a diffuse retinal detachment (RD), coming from the nasal aspect of the retina, surrounding the optic nerve, beneath the foveola, and finally accumulating in the inferior retina (Figure 1A). Fluorescein angiography showed diffuse retinal pigment epitheliopathy with focal granular hyperfluoNone of the authors has financial or proprietary interest in any material or method mentioned. Reprint requests: Javier Montero, MD, Avenida Tres Cruces, no. 2, 46014 Valencia, Spain; e-mail: [email protected]
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CHRONIC LEUKEMIA AND SERIOUS DETACHMENT
Fig. 1. A, Color fundus picture of the left eye showing a serous retinal detachment and a few intraretinal hemorrhages. B, Fluorescein angiography showing a choroidal blockage in the lower retina. In the nasal aspect of the retina, there is diffuse retinal pigment epitheliopathy with focal granular hyperfluorescence corresponding to window defects. C, Optical coherence tomography illustrates a serous retinal detachment with a large amount of fluid beneath the retinal pigment epithelium. D, Ocular ultrasound shows a serous RD with a subtle choroidal thickening that might be caused by leukemic choroidal infiltration.
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However, the leukocyte count was 124,000/mm3 (99% blasts). The patient was ultimately diagnosed as having Philadelphia chromosome–positive chronic myelogenous leukemia by the oncology department. Unfortunately, 6 weeks later, the patient died because of acute mesenteric ischemia. Discussion Chronic myelogenous leukemia is a hematopoietic systemic disorder that usually affects otherwise healthy people older than 40 years. The clinical features can be caused by either peripheral blood dyscrasia or choroidal infiltration.1 The most common findings for patients affected by leukemia are intraretinal hemorrhages, vein tortuosity, branch vein occlusions, and optic neuritis.2 In this case, the patient presented with an unusual serous RD. The differential diagnosis of a serous RD includes Haradas syndrome, choroidal hemangioma, malignant melanoma, uveal effusion, central serous retinopathy, optic pit syndrome, preeclampsia, Coats disease, metastatic neoplasm, lymphoma, Waldenstrom macroglobulinemia, and pulmonary hypertension among others. However, in our case, RD might be caused by choroidal infiltration by leukemic cells, leading to multiple retinal ischemias. Fluorescein angiography showed multifocal hyperfluorescence beneath the RD, and the ocular ultrasounds showed choroidal thickening; these findings are typical for leukemic choroidal infiltration.3 Serous RD is an uncommon feature among leukemia manifestations.4 Chronic leukemia may
remain asymptomatic until a blastic phase appears. Serous RD has been described as the initial symptom of other acute leukemias, but it has not been previously reported as the initial symptom for Philadelphia chromosome-positive chronic myelogenous leukemia.1-5 In this case, prompt recognition of the disease led to early systemic chemotherapy. Unfortunately, we do not have any follow-up with this patient. Key words: blastic phase, chronic myelogenous leukemia, optical coherence tomography, Philadelphia chromosome, serous retinal detachment. References 1. Miyamoto K, Kashii S, Honda Y. Serous retinal detachment caused by leukemic choroidal infiltration during complete remission. Br J Ophthalmol 2000;84:1318–1319. 2. Yoshida A, Kawano Y, Eto T, et al. Serous retinal detachment in an elderly patient with Philadelphia-chromosome-positive acute lymphoblastic leukemia. Am J Ophthalmol 2005;139: 348–349. 3. Stewart MW, Gitter KA, Cohen G. Acute leukemia presenting as a unilateral exudative retinal detachment. Retina 1989;9: 110–114. 4. Riss JM, Kaplanski G, Righini-Chossegros M, Harle JR, Escoffier P, Saracco JB. [Bilateral serous detachment of neuroepithelium of the posterior pole disclosing acute leukemia]. J Fr Ophtalmol 1990;13:563–568. 5. Schmiegelow K, Scherfig E, Prause JU, Jensen OA. Isolated leukemic choroidal relapse in a child with acute lymphoblastic leukemia one year off therapy, diagnosed through transvitreal retino-choroidal biopsy. Acta Ophthalmol (Copenh) 1988;66: 33–37.
Purpose: The purpose of this study was to report a case of diffuse unilateral retinal detachment as an initial symptom of chronic myelogenous leukemia in a 35-year-old woman. Methods: This is a case report of a 35-year-old white woman who presented with painless acute visual loss in her left eye. Results: A 35-year-old white woman presented with acute visual loss in her left eye. She had no systemic or ophthalmological medical history. Slit-lamp examination was normal in both eyes. Fundus examination of the left eye showed not only multiple retinal hemorrhages but also a diffuse serous retinal detachment affecting the nasal aspect of the retina and the posterior pole. The leukocyte count was 124,000/mm3 (99% blasts), and the Philadelphia chromosome was positive, an abnormality that is associated with chronic myelogenous leukemia. Conclusion: Serous retinal detachment is an uncommon feature among leukemias. Chronic myelogenous leukemia may remain asymptomatic until a blastic phase occurs. Prompt recognition of the disease leads to early systemic chemotherapy and may help to restore visual function. RETINAL CASES & BRIEF REPORTS 4:394–396, 2010
rescence corresponding to window defects and blockage caused by retinal pigment epithelium atrophy and clumping with one or more areas of subtle continued leakage. During the later phases of the angiogram, these areas gradually enlarged and stained the adjacent subretinal and subretinal pigment epithelium fluid (Figure IB). Optical coherence tomography confirmed a serous RD with a great amount of fluid, measuring .1,000 mm (Figure 1C). A complete laboratory examination, including complete blood count, autoantibody (antineutrophil cytoplasmic antibodies [ANCA], antinuclear antibodies [ANA]), venereal disease research laboratory search, erythrocyte sedimentation rate, C-reactive protein, and Mantoux test, was performed. In addition, a chest x-ray, an ocular ultrasound, and a chest examination were performed. The chest x-ray and the chest examination showed no pathologic findings. The ocular ultrasound showed an RD with a subtle choroidal thickening (Figure ID). The Mantoux test was negative.
From the Department of Ophthalmology, Hospital General Universitario de Valencia, Valencia, Spain.
A
35-year-old white woman presented with painless acute visual loss in her left eye. Her bestcorrected visual acuity was 20/20 in the right eye and 20/40 in the left eye. She had no systemic or ophthalmological medical history. Slit-lamp examination was normal. Fundus examination of the left eye showed not only multiple retinal hemorrhages but also a diffuse retinal detachment (RD), coming from the nasal aspect of the retina, surrounding the optic nerve, beneath the foveola, and finally accumulating in the inferior retina (Figure 1A). Fluorescein angiography showed diffuse retinal pigment epitheliopathy with focal granular hyperfluoNone of the authors has financial or proprietary interest in any material or method mentioned. Reprint requests: Javier Montero, MD, Avenida Tres Cruces, no. 2, 46014 Valencia, Spain; e-mail: [email protected]
394
CHRONIC LEUKEMIA AND SERIOUS DETACHMENT
Fig. 1. A, Color fundus picture of the left eye showing a serous retinal detachment and a few intraretinal hemorrhages. B, Fluorescein angiography showing a choroidal blockage in the lower retina. In the nasal aspect of the retina, there is diffuse retinal pigment epitheliopathy with focal granular hyperfluorescence corresponding to window defects. C, Optical coherence tomography illustrates a serous retinal detachment with a large amount of fluid beneath the retinal pigment epithelium. D, Ocular ultrasound shows a serous RD with a subtle choroidal thickening that might be caused by leukemic choroidal infiltration.
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However, the leukocyte count was 124,000/mm3 (99% blasts). The patient was ultimately diagnosed as having Philadelphia chromosome–positive chronic myelogenous leukemia by the oncology department. Unfortunately, 6 weeks later, the patient died because of acute mesenteric ischemia. Discussion Chronic myelogenous leukemia is a hematopoietic systemic disorder that usually affects otherwise healthy people older than 40 years. The clinical features can be caused by either peripheral blood dyscrasia or choroidal infiltration.1 The most common findings for patients affected by leukemia are intraretinal hemorrhages, vein tortuosity, branch vein occlusions, and optic neuritis.2 In this case, the patient presented with an unusual serous RD. The differential diagnosis of a serous RD includes Haradas syndrome, choroidal hemangioma, malignant melanoma, uveal effusion, central serous retinopathy, optic pit syndrome, preeclampsia, Coats disease, metastatic neoplasm, lymphoma, Waldenstrom macroglobulinemia, and pulmonary hypertension among others. However, in our case, RD might be caused by choroidal infiltration by leukemic cells, leading to multiple retinal ischemias. Fluorescein angiography showed multifocal hyperfluorescence beneath the RD, and the ocular ultrasounds showed choroidal thickening; these findings are typical for leukemic choroidal infiltration.3 Serous RD is an uncommon feature among leukemia manifestations.4 Chronic leukemia may
remain asymptomatic until a blastic phase appears. Serous RD has been described as the initial symptom of other acute leukemias, but it has not been previously reported as the initial symptom for Philadelphia chromosome-positive chronic myelogenous leukemia.1-5 In this case, prompt recognition of the disease led to early systemic chemotherapy. Unfortunately, we do not have any follow-up with this patient. Key words: blastic phase, chronic myelogenous leukemia, optical coherence tomography, Philadelphia chromosome, serous retinal detachment. References 1. Miyamoto K, Kashii S, Honda Y. Serous retinal detachment caused by leukemic choroidal infiltration during complete remission. Br J Ophthalmol 2000;84:1318–1319. 2. Yoshida A, Kawano Y, Eto T, et al. Serous retinal detachment in an elderly patient with Philadelphia-chromosome-positive acute lymphoblastic leukemia. Am J Ophthalmol 2005;139: 348–349. 3. Stewart MW, Gitter KA, Cohen G. Acute leukemia presenting as a unilateral exudative retinal detachment. Retina 1989;9: 110–114. 4. Riss JM, Kaplanski G, Righini-Chossegros M, Harle JR, Escoffier P, Saracco JB. [Bilateral serous detachment of neuroepithelium of the posterior pole disclosing acute leukemia]. J Fr Ophtalmol 1990;13:563–568. 5. Schmiegelow K, Scherfig E, Prause JU, Jensen OA. Isolated leukemic choroidal relapse in a child with acute lymphoblastic leukemia one year off therapy, diagnosed through transvitreal retino-choroidal biopsy. Acta Ophthalmol (Copenh) 1988;66: 33–37.
