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A Rare Association of Retinoblastoma With Incontinentia Pigmenti.

from compromised blood flow in >50% of splenic parenchyma, secondary to massive sickling.1 As Perada et al mentioned, it is typically encountered among patients with sickle cell trait/variants following an infection, postoperative stress, or exposure to high altitude. Persistence of an adequate spleen size among SCD heterozygous patients helps them retain some organ function, but also makes them more susceptible to MSI compared with homozygous patients, in whom autosplenectomy usually has occurred until the age of 5 years due to small, repetitive, silent infarcts.2 Interestingly, our patient was homozygous despite moderately increased hemoglobin S levels and her uneventful past medical history, since according to her mother, the child had never been hospitalized before in the past. In addition, despite the high prevalence of SCD, the Republic of Congo has not yet established any national newborn screening program for the disease. As no specific pathogen was identified, we hypothesized that dehydration due to infectious gastroenteritis was the trigger for MSI. One could say that the MSI was a result of hypertransfusion practice; however, the toddler had received only 1 unit of PRBC before deteriorating. To our knowledge, this is the first report wherein homozygous SCD presented initially with MSI.