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Pediatr Transplant. 2019 Mar;23(2):e13344. doi: 10.1111/petr.13344. Epub 2019 Jan 20.

Purtscher-like retinopathy: A rare presentation of hematopoietic stem cell transplant-associated thrombotic microangiopathy.

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Division of Pediatric Hematology Oncology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.
Department of Ophthalmology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.


Hematopoietic stem cell transplant (HSCT)-associated (TA) thrombotic microangiopathy (TMA) is an acquired disorder and a potentially life-threatening complication after allogeneic HSCT. TA-TMA causes endothelial damage and results in micro-thrombi in capillaries and arterioles. Early detection and treatment of complications associated with TA-TMA might improve outcomes. Purtscher-like retinopathy (PLR) is associated with micro-thrombi that occlude the retinal arteries and cause retinal injury. PLR has been associated with multiple entities, including HUS and TTP, but has not previously been described in the setting of TA-TMA. Here, we describe an 18-year-old male who underwent a mismatched unrelated donor HSCT for relapsed acute lymphoblastic leukemia. The patient was diagnosed with TA-TMA based on standard defined criteria. He presented with acute onset of blurred vision with findings of multiple white retinal patches, retinal hemorrhages, and macular edema, thought initially to be hypertensive retinopathy. However, on further evaluation using fluorescein angiography and optical coherence tomography, the diagnosis was determined to be PLR. The patient was treated with intravitreal steroid injections (triamcinolone acetonide) with dramatic improvement of vision. The aim of this report is to make clinicians aware of PLR as a potential ocular complication associated with TA-TMA and that prompt intervention might reverse visual impairment.


hematopoietic stem cell transplant; retinopathy; transplant associated thrombotic microangiopathy

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