Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease

Owusu‐Ofori, Shirley; Hirst, Ceri

doi:10.1002/14651858.cd003425

Cited by 15 publications

(5 citation statements)

References 18 publications

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“…The crisis resolved only after splenectomy. While splenectomy may be indicated in SCA patients who develop splenic sequestration or hypersplenism, its role in the prevention or treatment of HS is unknown.…”

Section: Discussionmentioning

confidence: 74%

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

et al. 2015

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Section: Discussionmentioning

confidence: 74%

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

et al. 2015

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“…It is a severe event, either for the possible fatal consequences or for the risk of following recurrence. It is usually managed with splenectomy, a recognized treatment in transfusion‐dependent patients with thalassemia major, except for young patients who receive a chronic transfusion program . Although the number of patients enrolled does not allow us to compare our data to those reported in the literature for SS/β° patients, the events in our cohort are relevant, pointing to a not‐so mild clinical course.…”

Section: Discussionmentioning

confidence: 84%

HbS/β+ thalassemia: Really a mild disease? A National survey from the AIEOP Sickle Cell Disease Study Group with genotype‐phenotype correlation

Notarangelo

Agostini

Casale

et al. 2019

European J of Haematology

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Objectives HbS/β+ patients’ presence in Italy increased due to immigration; these patients are clinically heterogeneous, and specific guidelines are lacking. Our aim is to describe a cohort of HbS/β+ patients, with genotype‐phenotype correlation, in order to offer guidance for clinical management of such patients. Methods Retrospective cohort study of HbS/β+ patients among 15 AIEOP Centres. Results A total of 41 molecularly confirmed S/β+ patients were enrolled (1‐55 years, median 10.9) and classified on β+ mutation: IVS‐I‐110, IVS‐I‐6, promoter, and “others.” Prediagnostic events included VOC 16/41 (39%), ACS 6/41 (14.6%), sepsis 3/41 (3.7%), and avascular necrosis 3/41 (7,3%). Postdiagnostic events were VOC 22/41 (53.6% %), sepsis 4/41 (9.7%), ACS 4/41 (9.7%), avascular necrosis 3/41 (7.3%), aplastic crisis 2/41 (4.8%), stroke 1/41 (2.4%), ACS 1/41 (2.4%), and skin ulcerations 1/41 (2.4%). The IVS‐I‐110 group presented the lowest median age at first SCD‐related event (P = .02 vs promoter group) and the higher median number of severe events/year (0.26 events/patient/year) (P = .01 vs IVS‐I‐6 and promoter groups). Promoter group presented a specific skeletal phenotype. Treatment regimen applied was variable among the centers. Conclusions HbS/β+ is not always a mild disease. Patients with IVS‐I‐110 mutation could benefit from a standard of care like SS and S/β° patients. Standardization of treatment is needed.

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“…However, as there is a lack of evidence from the randomized control trials showing that splenectomy improves survival and decreases morbidity in patients with sickle cell disease, hence splenectomy was not considered. 7 …”

Section: Discussionmentioning

confidence: 99%

Bochdalek Hernia and Partial Diaphragmatic Agenesis: Pedicled Intercostal Muscle Flap and Mesh Repair in a Young Adult with Sickle Cell Disease

et al. 2021

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Congenital Bochdalek hernia (BH) in an adult is rare and has an unusual presentation. They are confined to the pediatric age group with an incidence of 1:3,000 live births. It rarely persists asymptomatic until adulthood. Surgical repair by thoracic, abdominal, or thoraco-abdominal approach is the treatment of choice with diaphragmatic reconstruction in associated diaphragmatic agenesis. With only 10 cases of BH with partial diaphragmatic agenesis reported to date, we discuss the rarity, unusual presentation, and management of BH in a young adult with sickle cell disease that has not been reported in the literature.

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Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease

Cited by 15 publications

References 18 publications

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

Hyperhemolysis syndrome in patients with sickle cell anemia: report of three cases

HbS/β+ thalassemia: Really a mild disease? A National survey from the AIEOP Sickle Cell Disease Study Group with genotype‐phenotype correlation

Bochdalek Hernia and Partial Diaphragmatic Agenesis: Pedicled Intercostal Muscle Flap and Mesh Repair in a Young Adult with Sickle Cell Disease

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