Physician perspectives about the diagnosis and management of acute chest syndrome.

acute chest syndrome sickle cell disease transfusions

Journal

Transfusion
ISSN: 1537-2995
Titre abrégé: Transfusion
Pays: United States
ID NLM: 0417360

Informations de publication

Date de publication:
07 Oct 2024
Historique:
revised: 17 09 2024
received: 20 06 2024
accepted: 18 09 2024
medline: 7 10 2024
pubmed: 7 10 2024
entrez: 7 10 2024
Statut: aheadofprint

Résumé

Acute chest syndrome (ACS) is the leading cause of mortality, accounting for 25% of all deaths among individuals with sickle cell disease (SCD). There is a lack of evidence-based laboratory and clinical risk stratification guidelines for the diagnosis and management of ACS. To better understand physician practices for the management of ACS in the United States, we created an ACS Working Group including hematology and transfusion medicine physicians from four different SCD treatment centers in the United States. The working group created a physician survey that included physician demographics and ACS diagnostic criteria that they had to rate. The survey also included three case scenarios to assess physician attitudes about the management of ACS. Management options included supportive and preventive strategies in addition to transfusion therapy options. Out of 455 physicians who received the survey, 195 responded (response rate = 43%). The respondents were primarily hematology/oncology physicians. The responses showed wide variability among physicians in how diagnostic criteria for ACS are used and how physicians risk-stratify ACS patients in their practice. The responses also reflected variability in the use of transfusions for ACS. Based on our results, we conclude that ACS is diagnosed and managed inconsistently among expert physicians, especially in their transfusion practices due to a lack of consensus on risk stratification criteria. Our data suggest an urgent need for well-designed prospective studies to provide evidence-based guidelines and minimize management variability among physicians who care for individuals with SCD and ACS.

Sections du résumé

BACKGROUND BACKGROUND
Acute chest syndrome (ACS) is the leading cause of mortality, accounting for 25% of all deaths among individuals with sickle cell disease (SCD). There is a lack of evidence-based laboratory and clinical risk stratification guidelines for the diagnosis and management of ACS.
STUDY DESIGN AND METHODS METHODS
To better understand physician practices for the management of ACS in the United States, we created an ACS Working Group including hematology and transfusion medicine physicians from four different SCD treatment centers in the United States. The working group created a physician survey that included physician demographics and ACS diagnostic criteria that they had to rate. The survey also included three case scenarios to assess physician attitudes about the management of ACS. Management options included supportive and preventive strategies in addition to transfusion therapy options.
RESULTS RESULTS
Out of 455 physicians who received the survey, 195 responded (response rate = 43%). The respondents were primarily hematology/oncology physicians. The responses showed wide variability among physicians in how diagnostic criteria for ACS are used and how physicians risk-stratify ACS patients in their practice. The responses also reflected variability in the use of transfusions for ACS.
DISCUSSION CONCLUSIONS
Based on our results, we conclude that ACS is diagnosed and managed inconsistently among expert physicians, especially in their transfusion practices due to a lack of consensus on risk stratification criteria. Our data suggest an urgent need for well-designed prospective studies to provide evidence-based guidelines and minimize management variability among physicians who care for individuals with SCD and ACS.

Identifiants

pubmed: 39373054
doi: 10.1111/trf.18034
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© 2024 The Author(s). Transfusion published by Wiley Periodicals LLC on behalf of AABB.

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Auteurs

Neha Bhasin (N)

Department of Pediatrics, Division of Hematology, University of California San Francisco Benioff Children's Hospital, Oakland, California, USA.

Dana Marie LeBlanc (DM)

Louisiana State University Health Science Center, New Orleans, Louisiana, USA.

Sean Yates (S)

Division of Transfusion Medicine and Hemostasis, University of Texas, Dallas, Texas, USA.

Quentin Eichbaum (Q)

Transfusion Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.

An Pham (A)

Department of Pediatrics, UT Southwestern University, Dallas, Texas, USA.

Deva Sharma (D)

Transfusion Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.

Li Zhang (L)

Department of Medicine, Division of Hematology and Medical Oncology, University of California, San Francisco, California, USA.

Elliott P Vichinsky (EP)

Department of Pediatrics, Division of Hematology, University of California San Francisco Benioff Children's Hospital, Oakland, California, USA.

Ravi Sarode (R)

Division of Transfusion Medicine and Hemostasis, University of Texas, Dallas, Texas, USA.

Classifications MeSH