Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

James E. Louie, Caitlin J. Anderson, Katayoun Fayaz M. Fomani, Alonye Henry, Trevor Killeen, Narla Mohandas, Karina Yazdanbakhsh, John D Belcher, Gregory M Vercellotti, Patricia A. Shi

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

BACKGROUND: Depletion of haptoglobin (Hp) and hemopexin (Hx) with increase in free hemoglobin and heme are important etiologies of vaso-occlusive complications in sickle cell disease (SCD). This study is the first to show an association between clinical improvement in SCD and repletion of Hp and Hx by therapeutic plasma exchange (TPE) using plasma replacement. STUDY DESIGN AND METHODS: Thirteen fresh-frozen plasma (FFP) units derived from consecutive whole blood donations were thawed at 37°C after 10 months of storage; Hp and Hx concentrations immediately postthaw and after 5 days of refrigerated storage were analyzed by enzyme-linked immunosorbent assay (ELISA). All SCD patients presenting to a single institution over a 2-year period with acute multiorgan failure syndrome resistant to red blood cell exchange (RCE) were treated with TPE with FFP replacement; concentrations of Hp, Hx, and heme were evaluated before and after TPE by ELISA. RESULTS: Plasma concentrations of Hp and Hx decreased approximately 20% (p ≤ 0.002) after 5 days of refrigerated storage. Significant mean fold increases after TPE of 10 for Hp (p < 0.005) and seven for Hx (p < 0.003) and a 30% mean decrease in heme concentrations (p = 0.07) were noted in association with clinical improvement (three patients), whereas minimal increases in Hp and Hx were associated with continued clinical deterioration in one patient. CONCLUSION: Fresh-frozen plasma rather than thawed plasma is optimal for Hp and Hx replacement. Patient data are consistent with Hp and Hx increases via TPE limiting clinical toxicity of worsened hemolysis associated with severe vaso-occlusive complications refractory to RCE in SCD.

Original languageEnglish (US)
Pages (from-to)470-479
Number of pages10
JournalTransfusion
Volume58
Issue number2
DOIs
StatePublished - Feb 1 2018

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Hemopexin
Plasma Exchange
Haptoglobins
Sickle Cell Anemia
Heme
Erythrocytes
Therapeutics
Enzyme-Linked Immunosorbent Assay
Hemolysis
Blood Donors
Hemoglobins

PubMed: MeSH publication types

  • Case Reports
  • Journal Article
  • Research Support, Non-U.S. Gov't

Cite this

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease. / Louie, James E.; Anderson, Caitlin J.; Fayaz M. Fomani, Katayoun; Henry, Alonye; Killeen, Trevor; Mohandas, Narla; Yazdanbakhsh, Karina; Belcher, John D; Vercellotti, Gregory M; Shi, Patricia A.

In: Transfusion, Vol. 58, No. 2, 01.02.2018, p. 470-479.

Research output: Contribution to journalArticle

Louie, James E. ; Anderson, Caitlin J. ; Fayaz M. Fomani, Katayoun ; Henry, Alonye ; Killeen, Trevor ; Mohandas, Narla ; Yazdanbakhsh, Karina ; Belcher, John D ; Vercellotti, Gregory M ; Shi, Patricia A. / Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease. In: Transfusion. 2018 ; Vol. 58, No. 2. pp. 470-479.
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abstract = "BACKGROUND: Depletion of haptoglobin (Hp) and hemopexin (Hx) with increase in free hemoglobin and heme are important etiologies of vaso-occlusive complications in sickle cell disease (SCD). This study is the first to show an association between clinical improvement in SCD and repletion of Hp and Hx by therapeutic plasma exchange (TPE) using plasma replacement. STUDY DESIGN AND METHODS: Thirteen fresh-frozen plasma (FFP) units derived from consecutive whole blood donations were thawed at 37°C after 10 months of storage; Hp and Hx concentrations immediately postthaw and after 5 days of refrigerated storage were analyzed by enzyme-linked immunosorbent assay (ELISA). All SCD patients presenting to a single institution over a 2-year period with acute multiorgan failure syndrome resistant to red blood cell exchange (RCE) were treated with TPE with FFP replacement; concentrations of Hp, Hx, and heme were evaluated before and after TPE by ELISA. RESULTS: Plasma concentrations of Hp and Hx decreased approximately 20{\%} (p ≤ 0.002) after 5 days of refrigerated storage. Significant mean fold increases after TPE of 10 for Hp (p < 0.005) and seven for Hx (p < 0.003) and a 30{\%} mean decrease in heme concentrations (p = 0.07) were noted in association with clinical improvement (three patients), whereas minimal increases in Hp and Hx were associated with continued clinical deterioration in one patient. CONCLUSION: Fresh-frozen plasma rather than thawed plasma is optimal for Hp and Hx replacement. Patient data are consistent with Hp and Hx increases via TPE limiting clinical toxicity of worsened hemolysis associated with severe vaso-occlusive complications refractory to RCE in SCD.",
author = "Louie, {James E.} and Anderson, {Caitlin J.} and {Fayaz M. Fomani}, Katayoun and Alonye Henry and Trevor Killeen and Narla Mohandas and Karina Yazdanbakhsh and Belcher, {John D} and Vercellotti, {Gregory M} and Shi, {Patricia A.}",
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T1 - Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

AU - Louie, James E.

AU - Anderson, Caitlin J.

AU - Fayaz M. Fomani, Katayoun

AU - Henry, Alonye

AU - Killeen, Trevor

AU - Mohandas, Narla

AU - Yazdanbakhsh, Karina

AU - Belcher, John D

AU - Vercellotti, Gregory M

AU - Shi, Patricia A.

PY - 2018/2/1

Y1 - 2018/2/1

N2 - BACKGROUND: Depletion of haptoglobin (Hp) and hemopexin (Hx) with increase in free hemoglobin and heme are important etiologies of vaso-occlusive complications in sickle cell disease (SCD). This study is the first to show an association between clinical improvement in SCD and repletion of Hp and Hx by therapeutic plasma exchange (TPE) using plasma replacement. STUDY DESIGN AND METHODS: Thirteen fresh-frozen plasma (FFP) units derived from consecutive whole blood donations were thawed at 37°C after 10 months of storage; Hp and Hx concentrations immediately postthaw and after 5 days of refrigerated storage were analyzed by enzyme-linked immunosorbent assay (ELISA). All SCD patients presenting to a single institution over a 2-year period with acute multiorgan failure syndrome resistant to red blood cell exchange (RCE) were treated with TPE with FFP replacement; concentrations of Hp, Hx, and heme were evaluated before and after TPE by ELISA. RESULTS: Plasma concentrations of Hp and Hx decreased approximately 20% (p ≤ 0.002) after 5 days of refrigerated storage. Significant mean fold increases after TPE of 10 for Hp (p < 0.005) and seven for Hx (p < 0.003) and a 30% mean decrease in heme concentrations (p = 0.07) were noted in association with clinical improvement (three patients), whereas minimal increases in Hp and Hx were associated with continued clinical deterioration in one patient. CONCLUSION: Fresh-frozen plasma rather than thawed plasma is optimal for Hp and Hx replacement. Patient data are consistent with Hp and Hx increases via TPE limiting clinical toxicity of worsened hemolysis associated with severe vaso-occlusive complications refractory to RCE in SCD.

AB - BACKGROUND: Depletion of haptoglobin (Hp) and hemopexin (Hx) with increase in free hemoglobin and heme are important etiologies of vaso-occlusive complications in sickle cell disease (SCD). This study is the first to show an association between clinical improvement in SCD and repletion of Hp and Hx by therapeutic plasma exchange (TPE) using plasma replacement. STUDY DESIGN AND METHODS: Thirteen fresh-frozen plasma (FFP) units derived from consecutive whole blood donations were thawed at 37°C after 10 months of storage; Hp and Hx concentrations immediately postthaw and after 5 days of refrigerated storage were analyzed by enzyme-linked immunosorbent assay (ELISA). All SCD patients presenting to a single institution over a 2-year period with acute multiorgan failure syndrome resistant to red blood cell exchange (RCE) were treated with TPE with FFP replacement; concentrations of Hp, Hx, and heme were evaluated before and after TPE by ELISA. RESULTS: Plasma concentrations of Hp and Hx decreased approximately 20% (p ≤ 0.002) after 5 days of refrigerated storage. Significant mean fold increases after TPE of 10 for Hp (p < 0.005) and seven for Hx (p < 0.003) and a 30% mean decrease in heme concentrations (p = 0.07) were noted in association with clinical improvement (three patients), whereas minimal increases in Hp and Hx were associated with continued clinical deterioration in one patient. CONCLUSION: Fresh-frozen plasma rather than thawed plasma is optimal for Hp and Hx replacement. Patient data are consistent with Hp and Hx increases via TPE limiting clinical toxicity of worsened hemolysis associated with severe vaso-occlusive complications refractory to RCE in SCD.

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DO - 10.1111/trf.14407

M3 - Article

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