American Society of Hematology 2019 guidelines for sickle cell disease: Cardiopulmonary and kidney disease

Robert I. Liem, Sophie Lanzkron, Thomas D. Coates, Laura DeCastro, Ankit A. Desai, Kenneth I. Ataga, Robyn T. Cohen, Johnson Haynes, Ifeyinwa Osunkwo, Jeffrey D. Lebensburger, James P. Lash, Theodore Wun, Madeleine Verhovsek, Elodie Ontala, Rae Blaylark, Fares Alahdab, Abdulrahman Katabi, Reem A. Mustafa

    Research output: Contribution to journalArticle

    Abstract

    Background: Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals. Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Methods: ASH formed a multidisciplinary guideline panel that included 2 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including performing systematic evidence reviews up to September 2017. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel agreed on 10 recommendations for screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Recommendations related to anticoagulation duration for adults with SCD and venous thromboembolism were also developed. Conclusions: Most recommendations were conditional due to a paucity of direct, high-quality evidence for outcomes of interest. Future research was identified, including the need for prospective studies to better understand the natural history of cardiopulmonary and renal disease, their relationship to patient-important outcomes, and optimal management.

    Original languageEnglish (US)
    Pages (from-to)3867-3897
    Number of pages31
    JournalBlood Advances
    Volume3
    Issue number23
    DOIs
    StatePublished - Jan 1 2019

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    Kidney Diseases
    Sickle Cell Anemia
    Guidelines
    Kidney
    Patient Advocacy
    Conflict of Interest
    Evidence-Based Practice
    Venous Thromboembolism
    Hematology
    Natural History
    Uncertainty
    Prospective Studies
    Delivery of Health Care
    Research

    ASJC Scopus subject areas

    • Hematology

    Cite this

    Liem, R. I., Lanzkron, S., Coates, T. D., DeCastro, L., Desai, A. A., Ataga, K. I., ... Mustafa, R. A. (2019). American Society of Hematology 2019 guidelines for sickle cell disease: Cardiopulmonary and kidney disease. Blood Advances, 3(23), 3867-3897. https://doi.org/10.1182/bloodadvances.2019000916

    American Society of Hematology 2019 guidelines for sickle cell disease : Cardiopulmonary and kidney disease. / Liem, Robert I.; Lanzkron, Sophie; Coates, Thomas D.; DeCastro, Laura; Desai, Ankit A.; Ataga, Kenneth I.; Cohen, Robyn T.; Haynes, Johnson; Osunkwo, Ifeyinwa; Lebensburger, Jeffrey D.; Lash, James P.; Wun, Theodore; Verhovsek, Madeleine; Ontala, Elodie; Blaylark, Rae; Alahdab, Fares; Katabi, Abdulrahman; Mustafa, Reem A.

    In: Blood Advances, Vol. 3, No. 23, 01.01.2019, p. 3867-3897.

    Research output: Contribution to journalArticle

    Liem, RI, Lanzkron, S, Coates, TD, DeCastro, L, Desai, AA, Ataga, KI, Cohen, RT, Haynes, J, Osunkwo, I, Lebensburger, JD, Lash, JP, Wun, T, Verhovsek, M, Ontala, E, Blaylark, R, Alahdab, F, Katabi, A & Mustafa, RA 2019, 'American Society of Hematology 2019 guidelines for sickle cell disease: Cardiopulmonary and kidney disease', Blood Advances, vol. 3, no. 23, pp. 3867-3897. https://doi.org/10.1182/bloodadvances.2019000916
    Liem, Robert I. ; Lanzkron, Sophie ; Coates, Thomas D. ; DeCastro, Laura ; Desai, Ankit A. ; Ataga, Kenneth I. ; Cohen, Robyn T. ; Haynes, Johnson ; Osunkwo, Ifeyinwa ; Lebensburger, Jeffrey D. ; Lash, James P. ; Wun, Theodore ; Verhovsek, Madeleine ; Ontala, Elodie ; Blaylark, Rae ; Alahdab, Fares ; Katabi, Abdulrahman ; Mustafa, Reem A. / American Society of Hematology 2019 guidelines for sickle cell disease : Cardiopulmonary and kidney disease. In: Blood Advances. 2019 ; Vol. 3, No. 23. pp. 3867-3897.
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    AU - Liem, Robert I.

    AU - Lanzkron, Sophie

    AU - Coates, Thomas D.

    AU - DeCastro, Laura

    AU - Desai, Ankit A.

    AU - Ataga, Kenneth I.

    AU - Cohen, Robyn T.

    AU - Haynes, Johnson

    AU - Osunkwo, Ifeyinwa

    AU - Lebensburger, Jeffrey D.

    AU - Lash, James P.

    AU - Wun, Theodore

    AU - Verhovsek, Madeleine

    AU - Ontala, Elodie

    AU - Blaylark, Rae

    AU - Alahdab, Fares

    AU - Katabi, Abdulrahman

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