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

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 b...

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Published in:Transfusion (Philadelphia, Pa.) Pa.), 2018-02, Vol.58 (2), p.470-479
Main Authors: 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.
Format: Article
Language:English
Subjects:
Adult
Apheresis
Blood
Blood & organ donations
Blood donors
Clinical deterioration
Cold storage
Complications
Detoxification
Enzyme-linked immunosorbent assay
Erythrocyte Transfusion
Erythrocytes
Etiology
Exchanging
Female
Haptoglobin
Heme
Heme - metabolism
Hemoglobin
Hemopexin
Humans
Male
Middle Aged
Multiple Organ Failure - blood
Multiple Organ Failure - therapy
Patients
Plasma
Plasma Exchange
Sickle cell disease
Toxicity
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container_title Transfusion (Philadelphia, Pa.)
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creator 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.
description 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 
doi_str_mv 10.1111/trf.14407
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Fomani, Katayoun ; Henry, Alonye ; Killeen, Trevor ; Mohandas, Narla ; Yazdanbakhsh, Karina ; Belcher, John D. ; Vercellotti, Gregory M. ; Shi, Patricia A.</creator><creatorcontrib>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.</creatorcontrib><description>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 &lt; 0.005) and seven for Hx (p &lt; 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.</description><identifier>ISSN: 0041-1132</identifier><identifier>EISSN: 1537-2995</identifier><identifier>DOI: 10.1111/trf.14407</identifier><identifier>PMID: 29193101</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>Adult ; Apheresis ; Blood ; Blood &amp; organ donations ; Blood donors ; Clinical deterioration ; Cold storage ; Complications ; Detoxification ; Enzyme-linked immunosorbent assay ; Erythrocyte Transfusion ; Erythrocytes ; Etiology ; Exchanging ; Female ; Haptoglobin ; Heme ; Heme - metabolism ; Hemoglobin ; Hemopexin ; Humans ; Male ; Middle Aged ; Multiple Organ Failure - blood ; Multiple Organ Failure - therapy ; Patients ; Plasma ; Plasma Exchange ; Sickle cell disease ; Toxicity</subject><ispartof>Transfusion (Philadelphia, Pa.), 2018-02, Vol.58 (2), p.470-479</ispartof><rights>2017 AABB</rights><rights>2017 AABB.</rights><rights>2018 AABB</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3537-1bdc1e500a67d6438f4fee1799bd23f61d1ed1747129410d92009388796fe3513</citedby><cites>FETCH-LOGICAL-c3537-1bdc1e500a67d6438f4fee1799bd23f61d1ed1747129410d92009388796fe3513</cites><orcidid>0000-0002-7954-0055</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29193101$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Louie, James E.</creatorcontrib><creatorcontrib>Anderson, Caitlin J.</creatorcontrib><creatorcontrib>Fayaz M. Fomani, Katayoun</creatorcontrib><creatorcontrib>Henry, Alonye</creatorcontrib><creatorcontrib>Killeen, Trevor</creatorcontrib><creatorcontrib>Mohandas, Narla</creatorcontrib><creatorcontrib>Yazdanbakhsh, Karina</creatorcontrib><creatorcontrib>Belcher, John D.</creatorcontrib><creatorcontrib>Vercellotti, Gregory M.</creatorcontrib><creatorcontrib>Shi, Patricia A.</creatorcontrib><title>Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease</title><title>Transfusion (Philadelphia, Pa.)</title><addtitle>Transfusion</addtitle><description>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 &lt; 0.005) and seven for Hx (p &lt; 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.</description><subject>Adult</subject><subject>Apheresis</subject><subject>Blood</subject><subject>Blood &amp; organ donations</subject><subject>Blood donors</subject><subject>Clinical deterioration</subject><subject>Cold storage</subject><subject>Complications</subject><subject>Detoxification</subject><subject>Enzyme-linked immunosorbent assay</subject><subject>Erythrocyte Transfusion</subject><subject>Erythrocytes</subject><subject>Etiology</subject><subject>Exchanging</subject><subject>Female</subject><subject>Haptoglobin</subject><subject>Heme</subject><subject>Heme - metabolism</subject><subject>Hemoglobin</subject><subject>Hemopexin</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Multiple Organ Failure - blood</subject><subject>Multiple Organ Failure - therapy</subject><subject>Patients</subject><subject>Plasma</subject><subject>Plasma Exchange</subject><subject>Sickle cell disease</subject><subject>Toxicity</subject><issn>0041-1132</issn><issn>1537-2995</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><recordid>eNp1kc2KFDEURoMoTju68AUk4EYXPZObVFU6S2kcFQYEGddFunKrO2MqKfOj08_kS5q2RkHBbELCyblf-Ah5DuwC6rrMcbyApmHyAVlBK-SaK9U-JCvGGlgDCH5GnqR0yxjjisFjcsYVKAEMVuTHViekCaPFRFOZ5xCz9Xt6wAmpwRzu7GgHnW3w9JvVNB8w6hlLtgOdnU6Tpng3HLTfI7We6qFkpFNx9UHca09HbV2JdcLRmxiqM2KyKWufaQ71YOjOhWDogM79ZUp2-OJwuTc2YY35lDwatUv47H4_J5-v3t5s36-vP777sH1zvR7E6fOwMwNgy5jupOkasRmbERGkUjvDxdiBATQgGwlcNcCM4owpsdlI1Y0oWhDn5NXinWP4WjDlfrLpFER7DCX1oCR0bculqujLf9DbUKKv6SqlGslF28lKvV6oIYaUIo79HO2k47EH1p8a7GuD_a8GK_vi3lh2E5o_5O_KKnC5AN-tw-P_Tf3Np6tF-RMukKfE</recordid><startdate>201802</startdate><enddate>201802</enddate><creator>Louie, James E.</creator><creator>Anderson, Caitlin J.</creator><creator>Fayaz M. 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Fomani, Katayoun</au><au>Henry, Alonye</au><au>Killeen, Trevor</au><au>Mohandas, Narla</au><au>Yazdanbakhsh, Karina</au><au>Belcher, John D.</au><au>Vercellotti, Gregory M.</au><au>Shi, Patricia A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease</atitle><jtitle>Transfusion (Philadelphia, Pa.)</jtitle><addtitle>Transfusion</addtitle><date>2018-02</date><risdate>2018</risdate><volume>58</volume><issue>2</issue><spage>470</spage><epage>479</epage><pages>470-479</pages><issn>0041-1132</issn><eissn>1537-2995</eissn><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 &lt; 0.005) and seven for Hx (p &lt; 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.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>29193101</pmid><doi>10.1111/trf.14407</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0002-7954-0055</orcidid></addata></record>
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ispartof Transfusion (Philadelphia, Pa.), 2018-02, Vol.58 (2), p.470-479
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1537-2995
language eng
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source Wiley-Blackwell Read & Publish Collection
subjects Adult
Apheresis
Blood
Blood & organ donations
Blood donors
Clinical deterioration
Cold storage
Complications
Detoxification
Enzyme-linked immunosorbent assay
Erythrocyte Transfusion
Erythrocytes
Etiology
Exchanging
Female
Haptoglobin
Heme
Heme - metabolism
Hemoglobin
Hemopexin
Humans
Male
Middle Aged
Multiple Organ Failure - blood
Multiple Organ Failure - therapy
Patients
Plasma
Plasma Exchange
Sickle cell disease
Toxicity
title Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease
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