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Guideline
. 2019 Dec 10;3(23):3867-3897.
doi: 10.1182/bloodadvances.2019000916.

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

Robert I Liem  1   2 , Sophie Lanzkron  3 , Thomas D Coates  4 , Laura DeCastro  5 , Ankit A Desai  6 , Kenneth I Ataga  7 , Robyn T Cohen  8 , Johnson Haynes  9 , Ifeyinwa Osunkwo  10 , Jeffrey D Lebensburger  11 , James P Lash  12 , Theodore Wun  13 , Madeleine Verhovsek  14 , Elodie Ontala  15 , Rae Blaylark  16 , Fares Alahdab  17 , Abdulrahman Katabi  17 , Reem A Mustafa  18
Affiliations
Guideline

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

Robert I Liem et al. Blood Adv. .

Erratum in

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.

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Conflict of interest statement

Conflict-of-interest disclosure: All authors were members of the guideline panel or members of the systematic review team or both. As such, they completed disclosure-of-interest forms, which were reviewed by ASH and are available as supplemental Files 2 and 3.

References

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