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Retinoblastoma: A fatal outcome of previously unreported association with sickle cell anaemia


MOJ Biology and Medicine
Farouk AG,1 Ibrahim HU,2 Usman B,3 Dada BJ,4 Ambe JP1

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Abstract

Retinoblastoma (RB) is the most common ocular malignancy. Presentation of this embryonal tumour in association with Sickle cell anaemia (SCA) has never been reported in the literature. The aetiology of RB remains unknown with unilateral disease occurring in about 60% nonhereditary form, and 15% hereditary. Here we present RB in association with SCA. A 6-year-old SCA developed progressive swelling of the left eye for 2-months. Physical examination of the eye showed proptosis and inability to move the eyeball, loss of vision and absent red-light reflex. Histopathology of enucleated eye macroscopically revealed a distorted eyeball. Microscopy revealed a section of a malignant neoplasm composing dense and loose masses of small round cells with hyperchromatic nuclei and scanty cytoplasm, cells were arranged in sheets with few Flexner-wintersteiner rosettes. Presentation of RB in a patient with SCA may lead to management challenges in our settings with the paucity of voluntary blood donors.

Keywords

embryonal, enucleation, malignant, retinoblastoma, sickle cell

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