When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Sickle cell anemia

Последний просмотренный: 16 Sep 2025
Last updated: 09 Jul 2025

Резюме

Определение

Анамнез и осмотр

Ключевые диагностические факторы

  • parent(s) diagnosed with sickle cell anemia, other sickle cell disease, or sickle cell trait
  • persistent pain in skeleton, chest, and/or abdomen
  • dactylitis
Полная информация

Другие диагностические факторы

  • high temperature
  • pneumonia-like syndrome
  • bone pain
  • visual floaters
  • tachypnea
  • failure to thrive
  • pallor
  • jaundice
  • tachycardia
  • lethargy
  • protuberant abdomen, often with umbilical hernia
  • cardiac systolic flow murmur
  • maxillary hypertrophy with overbite
  • shock
Полная информация

Факторы риска

  • genetic
Полная информация

Диагностические исследования

Исследования, которые показаны в первую очередь

  • DNA-based assays
  • hemoglobin isoelectric focusing (Hb IEF)
  • cellulose acetate electrophoresis
  • high-performance liquid chromatography (HPLC) fractionation
  • hemoglobin solubility testing
  • peripheral blood smear
  • CBC and reticulocyte count
  • iron studies
Полная информация

Исследования, проведение которых нужно рассмотреть

  • pulse oximetry
  • plain x-rays of long bones
  • bacterial cultures
  • chest x-ray
Полная информация

Неотложные исследования

  • noninvasive prenatal testing (NIPT)

Алгоритм лечения

Острый

vaso-occlusive crisis

acute chest syndrome

ПРОДОЛЖЕНИЕ

chronic disease

Составители

Авторы

Sophie Lanzkron, MD, MHS

Director

Sickle Cell Center for Adults

Associate Professor of Medicine and Oncology

Johns Hopkins Medicine

Baltimore

MD

Раскрытие информации

SL declares consultancy (Bluebird bio, Novo Nordisk, Pfizer, Novartis, Magenta); honoraria (Novartis); research funding (Imara, Novartis, GBT, Takeda, CSL-Behring, HRSA, PCORI, MD CHRC); stocks (Pfizer, Teva). SL is on the executive board of the National Alliance for Sickle Cell Centers (uncompensated).

Выражение благодарностей

Dr Sophie Lanzkron would like to gratefully acknowledge Dr Channing Paller, a previous contributor to this topic.

Раскрытие информации

CP declares that she has no competing interests.

Рецензенты

James Bradner, MD

Instructor in Medicine

Division of Hematologic Neoplasia

Dana-Farber Cancer Institute

Boston

MA

Раскрытие информации

JB declares that he has no competing interests.

Adrian Stephens, MB BS, MD, FRCPath

Consultant Haematologist

University College London Hospitals

London

UK

Раскрытие информации

AS declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Список литературы

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Основные статьи

Bain BJ, Daniel Y, Henthorn J, et al. Significant haemoglobinopathies: a guideline for screening and diagnosis. Br J Haematol. 2023 Jun;201(6):1047-65.Полный текст  Аннотация

National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. Sep 2014 [internet publication].Полный текст

DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-88.Полный текст  Аннотация

Chou ST, Alsawas M, Fasano RM, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-55.Полный текст  Аннотация

Trompeter S, Massey E, Robinson S, et al. Position paper on International Collaboration for Transfusion Medicine (ICTM) Guideline 'Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline'. Br J Haematol. 2020 May;189(3):424-27.Полный текст  Аннотация

Статьи, указанные как источники

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

Использование этого контента попадает под действие нашего заявления об отказе от ответственности