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PHOTO ESSAY
Year : 2019  |  Volume : 67  |  Issue : 12  |  Page : 2049-2051

“String of beads” appearance on fundus fluorescein angiography as a clinical clue for leukemia-related proliferative retinopathy


1 Department of Vitreo-Retina, Narayana Nethralaya, Bangalore, Karnataka, India
2 Department of Uveitis and Ocular Immunology, Narayana Nethralaya, Bangalore, Karnataka, India
3 Department of Cararact and Refractive Lens Surgery, Narayana Nethralaya, Bangalore, Karnataka, India

Date of Submission30-Jan-2019
Date of Acceptance08-Aug-2019
Date of Web Publication22-Nov-2019

Correspondence Address:
Dr. Padmamalini Mahendradas
Department of Uveitis and Ocular Immunology, Narayana Nethralaya, Chord Road, Rajajinagar, Bangalore -560 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_233_19

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Keywords: Fundus fluorescein angiography, leukemia, leukemic retinopathy, proliferative retinopathy, Roth spots


How to cite this article:
Priya B V, Jain K, Mahendradas P, Shetty BK. “String of beads” appearance on fundus fluorescein angiography as a clinical clue for leukemia-related proliferative retinopathy. Indian J Ophthalmol 2019;67:2049-51

How to cite this URL:
Priya B V, Jain K, Mahendradas P, Shetty BK. “String of beads” appearance on fundus fluorescein angiography as a clinical clue for leukemia-related proliferative retinopathy. Indian J Ophthalmol [serial online] 2019 [cited 2019 Dec 14];67:2049-51. Available from: http://www.ijo.in/text.asp?2019/67/12/2049/271381



A 58-year-old male presented with blurring of vision and floaters in the right eye (OD) since 1 month. His best-corrected visual acuity was 20/20, N6 OD and 20/20, N6 left eye (OS). He was a known case of type II diabetes mellitus under oral hypoglycemic agents with controlled sugar levels. He gave history of 9 kg weight loss in the past 2 months and an episode of fever 2 months ago. His anterior segment examination was normal in both the eyes. Fundus examination showed presence of bilateral multiple retinal hemorrhages, Roth spots, perivascular sheathing, and retinal infiltrates with vitreous haemorrhage in the right eye [Figure 1] and [Figure 2]. With differential diagnosis of infectious retinal vasculitis, occlusive vascular retinopathy, and masquerades, fundus fluorescein angiography (FFA) and systemic work up were advised. FFA revealed bilateral multiple hyperfluorescent dots, leakage suggestive of neovascularization of the disc and elsewhere, multiple capillary nonperfusion areas [Figure 3] and [Figure 4], and a peculiar bumpy appearance of vessels similar to a “string of beads” [Figure 5]. This appearance of vessels was seen in arteries, arterioles, and capillaries but was most prominent in capillaries. On systemic work up, the patient was found to have leucocytosis (2,085,000/μL), erythrocyte sedimentation rate of 34 mm/h, C-reactive protein positive, and presence of myeloblasts and immature granulocytes on peripheral smear. Mantoux test,  Treponema pallidum Scientific Name Search emagglutination assay, HIV-1 and 2, and polymerase chain reaction for common viral genomes were negative. In view of leukocytosis, bone marrow biopsy was done, which revealed presence of Philadelphia translocation “t(9;22)(q34;q11).” He was diagnosed to have chronic myeloid leukemia (CML; chronic phase). The patient was subjected to laser pan-retinal photocoagulation and systemic chemotherapy in the form of T. Imatinib mesylate was started by the hemato-oncologist.
Figure 1: Colour fundus photograph of right eye showing vitreous haemorrhage with multiple retinal hemorrhages, Roth spots, perivascular sheathing, and retinal infiltrate

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Figure 2: Colour fundus photograph of left eye showing multiple retinal hemorrhages, Roth spots, perivascular sheathing, and retinal infiltrates

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Figure 3: Fundus fluorescein angiogram of right eye showing multiple hyperfluorescent dots, leakage suggestive of neovascularization of the disc and elsewhere, and multiple capillary nonperfusion areas

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Figure 4: Fundus fluorescein angiogram of left eye showing multiple hyperfluorescent dots, leakage suggestive of neovascularization of the disc and elsewhere, and multiple capillary nonperfusion areas

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Figure 5: Zoomed-in image of fundus fluorescein angiogram showing a peculiar bumpy appearance of vessels similar to a “string of beads” (solid red arrow)

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CML is a clonal myeloproliferative disorder of hematopoietic stem cells. Bilateral proliferative retinopathy as an initial presentation of CML has been described previously.[1],[2],[3],[4],[5] The “string of beads” term has been used to describe the radiological appearance of vessels in fibromuscular dysplasia[6] and as a radiological sign in small bowel obstruction.[7] In ophthalmology, the term has been used to describe the appearance of vitreous opacities in Candida endophthalmitis.[8] However, the term has not been used to describe the appearance of vessels on FFA yet. CML with leukocytosis leading to hyperviscosity may be an explanation for this particular appearance of vessels. This could be an important diagnostic imaging clue on FFA in cases of proliferative retinopathy secondary to leukemia. However, larger case series is required to confirm this observation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Macedo MS, Figueiredo AR, Ferreira NN, Barbosa IM, Furtado MF, Correia NF, et al. Bilateral proliferative retinopathy as the initial presentation of chronic myeloid leukemia. Middle East Afr J Ophthalmol 2013;20:3536.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Nobacht S, Vandoninck KF, Deutman AF, Klevering BJ. Peripheral retinal nonperfusion associated with chronic myeloid leukemia. Am J Ophthalmol 2003;135:404-6.  Back to cited text no. 2
    
3.
Mandava N, Costakos D, Bartlett HM. Chronic myelogenous leukemia manifested as bilateral proliferative retinopathy. Arch Ophthalmol 2005;123:576-7.  Back to cited text no. 3
    
4.
Morse PH, McCready JL. Peripheral retinal neovascularisation in chronic myelocytic leukemia. Am J Ophthalmol 1971;72:975-8.  Back to cited text no. 4
    
5.
Frank RN, Ryan SJ Jr. Peripheral retinal neovascularisation with chronic myelogenous leukemia. Arch Ophthalmol 1972;87:585-9.  Back to cited text no. 5
    
6.
Tegtmeyer CJ, Elson J, Glass TA, Ayers CR, Chevalier RL, Wellons HA Jr,et al. Percutaneous transluminal angioplasty: The treatment of choice for renovascular hypertension due to fibromuscular dysplasia. Radiology 1982;143:631-7.  Back to cited text no. 6
    
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Nevitt PC. The string of pearls sign. Radiology 2000;214:157-8.  Back to cited text no. 7
    
8.
Biju R, Sushil D, Georgy NK. Successful management of presumed Candida endogenous endophthalmitis with oral voriconazole. Indian J Ophthalmol 2009;57:306-8.  Back to cited text no. 8
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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