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The clinical spectrum of HbSC sickle cell disease-not a benign condition.
Nelson, M; Noisette, L; Pugh, N; Gordeuk, V; Hsu, L L; Wun, T; Shah, N; Glassberg, J; Kutlar, A; Hankins, J S; King, A A; Brambilla, D; Kanter, J.
Afiliación
  • Nelson M; Division of Hematology/Center for Sickle Cell Disease, Department of Medicine, University of Tennessee Health Science Center, Memphis, Tennessee, USA.
  • Noisette L; Division of Pediatric Hematology and Oncology, Children's Mercy Hospital, Kansas City, Missouri, USA.
  • Pugh N; Division of Biostatistics and Epidemiology, RTI International, North, Carolina, USA.
  • Gordeuk V; Division of Hematology and Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois, USA.
  • Hsu LL; Division of Pediatric Hematology-Oncology, University of Illinois at Chicago, Chicago, Illinois, USA.
  • Wun T; Division of Hematology and Oncology, Department of Medicine, UC Davis, Sacramento, California, USA.
  • Shah N; Pediatric Hematology/Oncology, Duke University, Durham, North Carolina, USA.
  • Glassberg J; Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, New York City, New York, USA.
  • Kutlar A; Sickle Cell Center, Augusta University, Augusta, Georgia, USA.
  • Hankins JS; Department of Global Pediatric Medicine and Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
  • King AA; Division of Pediatric Hematology and Oncology, Washington University School of Medicine, St. Louis, Missouri, USA.
  • Brambilla D; Division of Biostatistics and Epidemiology, RTI International, North, Carolina, USA.
  • Kanter J; Department of Medicine, University of Alabama, Birmingham, Alabama, USA.
Br J Haematol ; 205(2): 653-663, 2024 Aug.
Article en En | MEDLINE | ID: mdl-38898714
ABSTRACT
Sickle cell disease (SCD) includes a group of heterogenous disorders that result in significant morbidities. HbSS is the most common type of SCD and HbSC is the second most common type of SCD. The prevalence of HbSC disease in the United States and United Kingdom is ~1 in 7174 births and 1 in 6174 births respectively. Despite its frequency, however, HbSC disease has been insufficiently studied and was historically categorized as a more 'mild' form of SCD. We conducted this study of HbSC disease as part of the NHLBI funded Sickle Cell Disease Implementation Consortium (SCDIC). The SCDIC registry included 2282 individuals with SCD, ages 15-45 years of whom 502 (22%) had HbSC disease. Compared with people with sickle cell anaemia (SCA), the study found that people with HbSC disease had a higher frequency of splenomegaly (n (%) = 169 (33.7) vs. 392 (22.1)) and retinopathy (n (%) = 116 (23.1) vs. 189 (10.6)). A Many people with HbSC also had avascular necrosis (n (%) = 112 (22.3)), pulmonary embolism (n (%) = 43 (8.6)) and acute chest syndrome (n (%) = 228 (45.4)) demonstrating significant disease severity. HbSC disease is more clinically severe than was previously recognized and deserves additional evaluation and targeted treatments.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Anemia de Células Falciformes Límite: Adolescent / Adult / Female / Humans / Male / Middle aged País/Región como asunto: America do norte Idioma: En Revista: Br J Haematol Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Anemia de Células Falciformes Límite: Adolescent / Adult / Female / Humans / Male / Middle aged País/Región como asunto: America do norte Idioma: En Revista: Br J Haematol Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido