Transfusion practices and complications in thalassemia

Ashutosh Lal, Trisha E. Wong, Jennifer Andrews, Vinod V. Balasa, Jong Hee Chung, Craig M. Forester, Alan K. Ikeda, Siobán B. Keel, Monica B. Pagano, Geetha Puthenveetil, Sanjay J. Shah, Jennifer C. Yu, Elliott P. Vichinsky

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs. RESULTS: The centers followed 717 patients with β-thalassemia (314, 43.8%) or α-thalassemia (394, 55%). One-third (34.7%) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9% of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group. CONCLUSION: This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.

Original languageEnglish (US)
Pages (from-to)2826-2835
Number of pages10
JournalTransfusion
Volume58
Issue number12
DOIs
StatePublished - Dec 1 2018

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Thalassemia
Isoantibodies
Iron Overload
Long-Term Care
Blood Transfusion
Anemia
Iron
Erythrocytes
Quality of Life
Demography
Guidelines
Phenotype
Antigens
Survival
Therapeutics
Population
Surveys and Questionnaires

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Hematology

Cite this

Lal, A., Wong, T. E., Andrews, J., Balasa, V. V., Chung, J. H., Forester, C. M., ... Vichinsky, E. P. (2018). Transfusion practices and complications in thalassemia. Transfusion, 58(12), 2826-2835. https://doi.org/10.1111/trf.14875

Transfusion practices and complications in thalassemia. / Lal, Ashutosh; Wong, Trisha E.; Andrews, Jennifer; Balasa, Vinod V.; Chung, Jong Hee; Forester, Craig M.; Ikeda, Alan K.; Keel, Siobán B.; Pagano, Monica B.; Puthenveetil, Geetha; Shah, Sanjay J.; Yu, Jennifer C.; Vichinsky, Elliott P.

In: Transfusion, Vol. 58, No. 12, 01.12.2018, p. 2826-2835.

Research output: Contribution to journalArticle

Lal, A, Wong, TE, Andrews, J, Balasa, VV, Chung, JH, Forester, CM, Ikeda, AK, Keel, SB, Pagano, MB, Puthenveetil, G, Shah, SJ, Yu, JC & Vichinsky, EP 2018, 'Transfusion practices and complications in thalassemia', Transfusion, vol. 58, no. 12, pp. 2826-2835. https://doi.org/10.1111/trf.14875
Lal A, Wong TE, Andrews J, Balasa VV, Chung JH, Forester CM et al. Transfusion practices and complications in thalassemia. Transfusion. 2018 Dec 1;58(12):2826-2835. https://doi.org/10.1111/trf.14875
Lal, Ashutosh ; Wong, Trisha E. ; Andrews, Jennifer ; Balasa, Vinod V. ; Chung, Jong Hee ; Forester, Craig M. ; Ikeda, Alan K. ; Keel, Siobán B. ; Pagano, Monica B. ; Puthenveetil, Geetha ; Shah, Sanjay J. ; Yu, Jennifer C. ; Vichinsky, Elliott P. / Transfusion practices and complications in thalassemia. In: Transfusion. 2018 ; Vol. 58, No. 12. pp. 2826-2835.
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abstract = "BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs. RESULTS: The centers followed 717 patients with β-thalassemia (314, 43.8{\%}) or α-thalassemia (394, 55{\%}). One-third (34.7{\%}) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9{\%} of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group. CONCLUSION: This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.",
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AU - Forester, Craig M.

AU - Ikeda, Alan K.

AU - Keel, Siobán B.

AU - Pagano, Monica B.

AU - Puthenveetil, Geetha

AU - Shah, Sanjay J.

AU - Yu, Jennifer C.

AU - Vichinsky, Elliott P.

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N2 - BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs. RESULTS: The centers followed 717 patients with β-thalassemia (314, 43.8%) or α-thalassemia (394, 55%). One-third (34.7%) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9% of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group. CONCLUSION: This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.

AB - BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs. RESULTS: The centers followed 717 patients with β-thalassemia (314, 43.8%) or α-thalassemia (394, 55%). One-third (34.7%) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9% of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group. CONCLUSION: This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.

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