RETINAL Cases & Brief Reports:
Turaka, Kiran MD; Shields, Carol L MD; Bianciotto, Carlos MD; Shields, Jerry A MD
From the Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Support provided by the Retina Research Foundation of the Retina Society in Cape town, South Africa (C.L.S.); the Paul Kayser International Award of Merit in Retina Research, Houston TX (J.A.S.); a donation from Michael, Bruce, and Ellen Ratner, New York, NY (J.A.S. and C.L.S.); the Mellon Charitable Giving from the Martha W. Rogers Charitable Trust, Philadelphia, PA (C.L.S.); the LuEsther Mertz Retinal Research Foundation, New York, NY (C.L.S.); and the Eye Tumor Research Foundation, Philadelphia, PA (C.L.S. and J.A.S.).
The authors have no financial interest in this article.
Reprint requests: Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107; e-mail: firstname.lastname@example.org
Purpose: To report vitreous hemorrhage as the initial manifestation in idiopathic thrombocytopenic purpura, an uncommon presentation.
Methods: Case report.
Results: A 69-year-old white woman presented with diffuse vitreous hemorrhage as the initial manifestation of idiopathic thrombocytopenic purpura, 12 years after plaque radiotherapy for choroidal melanoma.
Conclusion: Unexplained and recurrent vitreous hemorrhage should be evaluated for idiopathic thrombocytopenic purpura.
Idiopathic or immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a decreased number of circulating platelets manifest as petechia, extravasation of blood into skin or mucous membranes (purpura), or spontaneous hemorrhage.1 Children and women are more commonly affected.1 This condition can have ocular manifestations including subcutaneous eyelid hemorrhage, submucosal conjunctival hemorrhage, and retinal hemorrhage.1 Despite the hemorrhagic tendency, vitreous hemorrhage is distinctly uncommon in this setting.2
We report a patient whose initial manifestation of ITP was vitreous hemorrhage after plaque radiotherapy of choroidal melanoma.
A 69-year-old woman noted sudden decreased vision in the right eye. She had been treated with 8,000-cGy plaque radiotherapy for a 3.2-mm-thick choroidal melanoma in the right eye 12 years ago. Visual acuity was counting fingers in the right eye and 20/20 in the left eye. The anterior segment findings were normal in both eyes. Fundus examination in the right eye disclosed a regressed choroidal melanoma scar of 8 mm in diameter and 1.4 mm in thickness and a diffuse vitreous hemorrhage (Figures 1, A and B). Fundus in the left eye was normal without retinal or vitreous hemorrhage.
In retrospect, the patient noted many years of skin bruising after light trauma and gum oozing after dental work. Blood evaluation disclosed normal leukocyte and erythrocyte counts, blood smear, and clotting factors. However, the platelet count was decreased to 6,000/μL (normal, 140,000–400,000/μL). A diagnosis of ITP was suspected, and oral prednisolone 1 mg/kg daily was started. After 2 days, the platelet count further decreased to 2,000/μL. Intravenous immunoglobulin 2 gm/kg was infused once, and the platelet count improved to 50,000/μL. Oral prednisolone was continued, and the platelet count maintained at 96,000/μL at 3 months and 181,000/μL at 6 months. By 6 months, the vitreous hemorrhage had spontaneously resolved and visual acuity was counting fingers because of radiation-induced macular edema (Figure 1C).
Idiopathic thrombocytopenic purpura is a hematologic condition whereby the number of circulating platelets decreases related to an autoimmune phenomenon. Pathogenesis in both acute and chronic ITP is considered to be decreased platelet surface antigens and increased antiplatelet antibodies.1 No single laboratory result or clinical finding establishes a diagnosis of ITP; therefore, it is a diagnosis of exclusion.1
In a clinical practice, the most common causes for vitreous hemorrhage include proliferative diabetic retinopathy, posterior vitreous detachment, and ocular trauma.3 Less common causes include retinal vein occlusion, proliferative sickle cell retinopathy, and age-related macular degeneration.3 Idiopathic thrombocytopenic purpura leading to vitreous hemorrhage is exquisitely rare.
In the medical literature, there are no large case series on the spectrum of eye findings in ITP but there have been mostly single case reports.4 Most reported cases with eye findings have an established diagnosis of ITP.4 Our case was different in that the main manifestation to stimulate investigation for ITP was the extensive vitreous hemorrhage. There were no signs of active radiation retinopathy in our patient. A single case of massive vitreous hemorrhage with thrombocytopenia to <2,000/μL was reported by Okuda et al.4 Intracranial hemorrhage with vitreous hemorrhage5 and retinal hemorrhages6 in patients with ITP has been reported. Massive subretinal hemorrhage in a patient with ITP and macular degeneration was described by Inoue et al.7 Idiopathic thrombocytopenic purpura–related severe anemia and thrombocytopenia with retinal hemorrhages have also been reported.2
Vitreous hemorrhage is not uncommon after plaque radiotherapy. In one series of 270 patients with plaque-irradiated choroidal melanoma, vitreous hemorrhage was found in 18% at 5-year follow-up.8 Most cases of vitreous hemorrhage occur within the first 2 years to 5 years. Our patient was unusual in that the vitreous hemorrhage was quite delayed at 12 years after plaque radiotherapy. An underlying abnormality was suspected because there was a history of bleeding of the skin and gums. After adequate immune suppression, our patient showed improvement in the platelet count with resolution of the vitreous hemorrhage without the need for vitrectomy. We recommend that the patients with recurrent unexplained bleeding episodes have careful evaluation for possible hemorrhagic diatheses.
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4. Okuda A, Inoue M, Shinoda K, Tsubota K. Massive bilateral vitreoretinal hemorrhage in patient with chronic refractory idiopathic thrombocytopenic purpura. Graefes Arch Clin Exp Ophthalmol 2005;243:1190–1193.
5. Frankel CA, Pastore DJ. Idiopathic thrombocytopenic purpura with intracranial hemorrhage and vitreous hemorrhage. Clin Pediatr 1990;29:725–728.
6. Shah PA, Yang SS, Fung WE. Idiopathic thrombocytopenic purpura with massive subretinal hemorrhage. Arch Ophthalmol 2005;23:1612–1613.
7. Inoue T, Yanagi Y, Tamaki Y, Kami J, Kato Y. Massive subretinal haemorrhage secondary to age-related macular degeneration in a patient with idiopathic thrombocytopenic purpura. Eye 2004;18:656–657.
8. Shields CL, Cater J, Shields JA, Chao A, et al. Combined plaque radiotherapy and transpupillary thermotherapy for choroidal melanoma: tumor control and treatment complications in 270 consecutive patients. Arch Ophthalmol 2002;120: 933–940.
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