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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Summary: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 
ISSN:0041-1132
1537-2995
DOI:10.1111/trf.14407