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Guideline Historical Article Journal Article Research Support, N.I.H., Extramural Ppublish 127 citations

PubMed: 31794601 DOI: 10.1182/bloodadvances.2019000916

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

Blood Adv 3(23):3867-3897

Authors: 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

Affiliations

  • Division of Hematology, Oncology and Stem Cell Transplant, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.
  • Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL.
  • Division of Adult Hematology, School of Medicine, Johns Hopkins University, Baltimore, MD.
  • Division of Hematology, Oncology and Blood and Marrow Transplantation, Children's Hospital of Los Angeles, Los Angeles, CA.
  • Division of Hematology/Oncology, University of Pittsburgh Medical Center, Pittsburgh, PA.
  • Krannert Institute of Cardiology, School of Medicine, Indiana University, Indianapolis, IN.
  • Center for Sickle Cell Disease, University of Tennessee Health Science Center, Memphis, TN.
  • Division of Pediatric Pulmonary and Allergy, Boston Medical Center, School of Medicine, Boston University, Boston, MA.
  • Division of Pulmonary Disease, College of Medicine, University of South Alabama, Mobile, AL.
  • Division of Hematology, The Levine Cancer Institute, Atrium Health, Charlotte, NC.
  • Division of Pediatric Hematology/Oncology, School of Medicine, University of Alabama at Birmingham, Birmingham, AL.
  • Division of Nephrology, Department of Medicine, University of Illinois at Chicago, Chicago, IL.
  • Division of Hematology and Oncology, School of Medicine, University of California Davis, Sacramento, CA.
  • Division of Hematology and Thromboembolism, McMaster University, Hamilton, ON, Canada.
  • Milwaukee, WI.
  • Minneapolis, MN.
  • Division of General Internal Medicine, Mayo Medical School, Rochester, MN; and.
  • Division of Nephrology and Hypertension, School of Medicine, University of Kansas, Kansas City, KS.

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.

MeSH

Kidney Diseases, Cells, Hematology, Anemia, Sickle Cell, Kidney, Diagnosis, Hematologic Diseases

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