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

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Scopus citations

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

ASJC Scopus subject areas

  • Hematology

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    Liem, R. I., Lanzkron, S., Coates, T. D., DeCastro, L., Desai, A. A., Ataga, K. I., Cohen, R. T., Haynes, J., Osunkwo, I., Lebensburger, J. D., Lash, J. P., Wun, T., Verhovsek, M., Ontala, E., Blaylark, R., Alahdab, F., Katabi, A., & 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