Sickle cell disease: a review for the internist

Pinto, Valeria; Balocco, Manuela; Quintino, Sabrina; Forni, Gianluca

doi:10.1007/s11739-019-02160-x

Cited by 67 publications

(59 citation statements)

References 80 publications

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“…It was with the help of radiological studies, ultrasound, and MRCP that we were able to see this patient's enlarged liver and biliary dilatation. This case demonstrated to us and the medical community as a whole that asymptomatic patients can present in acute hepatic sequestration crisis, and that one's threshold for conducting lab and imaging tests should be low, and not just based on the right upper quadrant abdominal pain that is classically expected on presentation [1,[3][4][5][6]. This patient was treated symptomatically with continued elevation in his hyperbilirubinemia, which eventually necessitated a full red blood cell transfusion exchange.…”

Section: Discussionmentioning

confidence: 99%

“…This increase in hematocrit is potentially dangerous and requires a watchful eye as hyperviscosity can increase the risk of heart failure, stroke, and even acute coronary syndrome, which would then benefit from phlebotomy [4,5]. It should be noted that a liver percutaneous biopsy is no longer recommended and has shown to lead to life-threatening hemorrhage and death in 28%-36% of cases as seen in United Kingdom patients [6][7][8].…”

Section: Introductionmentioning

confidence: 99%

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Asymptomatic Hepatic Sequestration with Extreme Hyperbilirubinemia in an Adult Homozygous Sickle Cell Patient

Raza

Faraji

Khan

et al. 2020

Cureus

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Asymptomatic Hepatic Sequestration with Extreme Hyperbilirubinemia in an Adult Homozygous Sickle Cell Patient

Raza

Faraji

Khan

et al. 2020

Cureus

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“…Die Sichelzellerkrankung (SZE) zählt zu den weltweit häufigsten und klinisch bedeutsamsten erblichen Hämoglobinopathien, für welche ca. 5 % der Weltbevölkerung Mutationsträger sind [48,59]. Pro Jahr sind ca.…”

Section: Epidemiologie Pathogenese Und Klinische Manifestation Der Sunclassified

Makulopathie bei Sichelzellerkrankung

et al. 2021

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Zusammenfassung Hintergrund Die Sichelzellerkrankung (SZE) ist eine hereditäre Hämoglobinopathie, die durch rezidivierende vasookklusive Episoden zur Mikrozirkulationsstörung verschiedener Organsysteme mit teils letalem Ausgang führt. Bei der okulären Manifestation der SZE ist am bekanntesten die periphere Sichelzellretinopathie (SZR). Unabhängig davon kann es bereits früh im Krankheitsverlauf zur Sichelzellmakulopathie (SZM) kommen. Methoden Review der internationalen und deutschsprachigen Literatur zur okulären Beteiligung bei SZE mit Fokus auf die SZR und SZM sowie Überblick über aktuelle systemische Therapieansätze bei SZE anlässlich der Vorstellung zweier Patienten mit HbSS-SZE. Ergebnis und Schlussfolgerung Im Gegensatz zur SZR ist die SZM mit temporaler Verdünnung der inneren Netzhautschichten erst in den letzten 5 Jahren mit der Einführung von SD-OCT und OCTA vermehrt in die Literatur eingegangen. Unabhängig vom Vorliegen einer SZR kann es immerhin bei etwa der Hälfte der Patienten bereits früh im Krankheitsverlauf zu einer SZM kommen. Das Krankheitsbild wird auch in Deutschland durch den Fortschritt der systemischen Therapiemöglichkeiten und aufgrund von Migration präsenter werden. Durch Wissen um diese Komplikation der SZE kann eine frühzeitige Diagnosestellung erfolgen und unnötige Diagnostik vermieden werden.

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“…These sickle cells are rigid and cause ischemia through blockage of the microvasculature, which causes the clinical manifestations. As the cells are destroyed or removed from circulation by macrophages, there is resulting oxidative stress and inflammation from release of free heme molecules, as well as hemolytic anemia 90 . The most common neurologic manifestation associated with SCD is ischemic stroke, however movement disorders are also described.…”

Section: Disorders Of Bleeding and Clottingmentioning

confidence: 99%

Movement Disorders and Hematologic Diseases

Patel

Hall

Eichenseer

et al. 2020

Movement Disord Clin Pract

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BackgroundMovement disorders can be associated with or caused by hematological abnormalities. The objective of this review is to highlight features that will aid in the clinician's recognition and treatment of these disorders.MethodsMESH terms relevant to movement disorders and hematologic diseases were searched to identify conditions included in this narrative, educational review.ResultsSeveral conditions were identified, and they were organized by hematologic categories to include red blood cell abnormalities, white blood cell abnormalities, disorders of clotting and bleeding, hematologic malignancies, and others.ConclusionsThis review will increase providers’ understanding of disorders that include movement disorders and hematologic abnormalities. Basic hematologic laboratories can aid in assessment of these disorders, to include complete blood count/hemogram and peripheral blood smear. Recognition is key, especially in the setting of underlying malignancy, vitamin deficiency, or other disorder in which treatment is available.

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Sickle cell disease: a review for the internist

Cited by 67 publications

References 80 publications

Asymptomatic Hepatic Sequestration with Extreme Hyperbilirubinemia in an Adult Homozygous Sickle Cell Patient

Asymptomatic Hepatic Sequestration with Extreme Hyperbilirubinemia in an Adult Homozygous Sickle Cell Patient

Makulopathie bei Sichelzellerkrankung

Movement Disorders and Hematologic Diseases

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