Look into my eyes: An unusual first presentation of sickle cell disease

Sood, Rupali; Jiramongkolchai, Kim; Streiff, Michael B.; González, Christopher; Shanbhag, Satish; Lanzkron, Sophie; Arévalo, J. Fernando; Naik, Rakhi P.

doi:10.1002/ajh.24787

Cited by 2 publications

(3 citation statements)

References 11 publications

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“…Microcytosis in the presence of normal iron stores is frequently the only abnormality suggestive of hemoglobinopathy in these instances. [6] Our patient was also positive for dengue NS1 antigen; however, the presence of severe anemia was not explained by dengue fever in absence of symptoms and signs of hemorrhage.…”

Section: Discussionmentioning

confidence: 43%

“…e case reports by Yaranal et al, [5] and Sood et al, [6] presented a 55-year-old patient with bilateral lower limb paraplegia, and a 46-year-old patient with fat embolism syndrome, respectively, in previously undiagnosed SCD. Similarly, our patient was also a 60-year-old female who had no history of crisis related to SCD, whereas sickle crisis is a usual presenting complaint of patients in early childhood and adolescence according to the literature.…”

Section: Discussionmentioning

confidence: 99%

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A rare presentation of sickle cell disease diagnosed for the first time in a 60-year-old female: A misdiagnosis or missed diagnosis

Raval

Rathod

2023

MEDINDIA

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Sickle cell disease (SCD) mainly manifests in early childhood. During the first 6 months of life, infants are largely protected by high levels of hemoglobin F; soon thereafter, the disease becomes evident in form of various crises such as vaso-occlusive crisis, hemolytic crisis, and acute chest syndrome, and in rare instances aplastic crisis. Therefore, the majority of patients with SCD are diagnosed in childhood or adulthood, whereas those with sickle cell trait usually remain asymptomatic. However, here, we are presenting a rare case in which a patient has been diagnosed to have SCD for the 1st time at the age of 60 years while being treated for dengue and severe anemia. Our case is one of the rare presentations of SCD, as she never required any blood transfusions even during childbirth and without any sickle crises; therefore, she might not be diagnosed with SCD till her presentation to our hospital. This indicates that advanced age should not be considered as an exclusion criterion for the suspecting and diagnosis of SCD. This case outlines the need for awareness and incorporation of screening for sickle cell anemia in childhood or adulthood as same thalassemia screening programs. The early diagnosis by screening programs will ensure appropriate treatment and therefore improve the quality of life in such patients.

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Section: Discussionmentioning

confidence: 43%

Section: Discussionmentioning

confidence: 99%

A rare presentation of sickle cell disease diagnosed for the first time in a 60-year-old female: A misdiagnosis or missed diagnosis

Raval

Rathod

2023

MEDINDIA

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“…Neovascularizing retinopathy involving vision loss is a severe complication of sickle cell disease (SCD). [1][2][3][4] Although more prevalent in HbSC disease (;43%), it also occurs in sickle cell anemia (;14%). 5 Sickle retinopathy fundamentally stems from occlusion of the peripheral retinal vasculature, leading to ischemiareperfusion injury, inflammation, and angiogenesis (neovascularization).…”

Section: Introductionmentioning

confidence: 99%

Morphine promotes neovascularizing retinopathy in sickle transgeneic mice

et al. 2019

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Neovascularizing retinopathy is a significant complication of sickle cell disease (SCD), occurring more frequently in HbSC than HbSS disease. This risk difference is concordant with a divergence of angiogenesis risk, as identified by levels of pro- vs anti-angiogenic factors in the sickle patient’s blood. Because our prior studies documented that morphine promotes angiogenesis in both malignancy and wound healing, we tested whether chronic opioid treatment would promote retinopathy in NY1DD sickle transgenic mice. After 10 to 15 months of treatment, sickle mice treated with morphine developed neovascularizing retinopathy to a far greater extent than either of the controls (sickle mice treated with saline and wild-type mice treated identically with morphine). Our dissection of the mechanistic linkage between morphine and retinopathy revealed a complex interplay among morphine engagement with its μ opioid receptor (MOR) on retinal endothelial cells (RECs); morphine-induced production of tumor necrosis factor α and interleukin-6 (IL-6), causing increased expression of both MOR and vascular endothelial growth factor receptor 2 (VEGFR2) on RECs; morphine/MOR engagement transactivating VEGFR2; and convergence of MOR, VEGFR2, and IL-6 activation on JAK/STAT3-dependent REC proliferation and angiogenesis. In the NY1DD mice, the result was increased angiogenesis, seen as neovascularizing retinopathy, similar to the retinal pathology occurring in humans with SCD. Therefore, we conclude that chronic opioid exposure, superimposed on the already angiogenic sickle milieu, might enhance risk for retinopathy. These results provide an additional reason for development and application of opioid alternatives for pain control in SCD.

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Look into my eyes: An unusual first presentation of sickle cell disease

Cited by 2 publications

References 11 publications

A rare presentation of sickle cell disease diagnosed for the first time in a 60-year-old female: A misdiagnosis or missed diagnosis

A rare presentation of sickle cell disease diagnosed for the first time in a 60-year-old female: A misdiagnosis or missed diagnosis

Morphine promotes neovascularizing retinopathy in sickle transgeneic mice

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