Retinal involvement in acute thrombotic thrombocytopenic purpura: a case report
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(2020) 20:456
CASE REPORT
Open Access
Retinal involvement in acute thrombotic thrombocytopenic purpura: a case report A. G. N. M. K. Bandara*
and Anne Thushara Matthias
Abstract Background: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening hematological condition associated with deficiency in ADAMTS13. Ocular manifestations associated with TTP are uncommon. Case presentation: Here we report a case where a 44 year old female patient with a history of symptomatic anemia and cotton wool appearance in retina during ophthalmological examination and subsequently, she was diagnosed to have TTP. The proper management with Therapeutic Plasma Exchange (TPE) and IV methylprednisolone improved the condition of the patient markedly. Conclusion: It concludes that even though the presence of cotton wool appearance in retina is a nonspecific sign it needs to be properly evaluated as there can be underlying serious illnesses as in our patient. Cotton wool spots can be an early sign of underlying retinal compromise and it should be identified early. Keywords: Cotton wool spots in retina, Thrombotic Thrombocytopenic Purpura, Therapeutic Plasma Exchange, Case report
Background Thrombotic Thrombocytopenic Purpura (TTP) is a rare, life threatening hematological disease characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia associated with microvascular platelet rich thrombi. The brain is the most affected organ due to ischemia in TTP as 60% of patients have neurological symptoms at presentation, which range from headache to stroke and coma [1]. Ocular manifestations related to TTP are usually uncommon and underestimated due to the fact that they are mainly detectable in the preterminal stage of TTP [2]. Ocular involvement reported are serous macular detachments, choriocapillaris occlusion due to fibrin-platelet thrombi, retinal or vitreous hemorrhages, optic disc edema, neovascularization of the disc, central retinal vein occlusion, anisocoria, diplopia and homonymous hemianopia [3]. According to the published literature this was reported * Correspondence: [email protected] Colombo South Teaching Hospital, Kalubowila, Dehiwala, Western Province, Sri Lanka
only in 14–20% of patients with TTP [4]. Here we report a case of TTP where the patient presents with a nonspecific headache and ocular manifestation of cotton wool spot in right fundus without any visual disturbances.
Case presentation A 44 -year-old previously healthy female was admitted with a history of generalized body weakness, malaise, exertional shortness of breath for one-week and episodic nonspecific headache for last one-month duration. There was no fever, urinary, bowel, respiratory symptoms or visual disturbances. Despite blood transfusion during her second pregnancy due to low Haemoglobin, her both pregnancies were uneventful. There were no suggestive features of autoimmune diseases or no bleeding manifestations. She had no significant past medical history or family history. On hospital admission, her Glas
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