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

Autor: Louie JE; Long Island Jewish Medical Center, Northwell Health, New Hyde Park, New York., Anderson CJ; Lindley F. Kimball Research Institute, New York Blood Center, New York, New York., Fayaz M Fomani K; Long Island Jewish Medical Center, Northwell Health, New Hyde Park, New York., Henry A; Lindley F. Kimball Research Institute, New York Blood Center, New York, New York., Killeen T; Department of Hematology, Oncology, and Transplantation, University of Minnesota Medical School, Minneapolis, Minnesota., Mohandas N; Lindley F. Kimball Research Institute, New York Blood Center, New York, New York., Yazdanbakhsh K; Lindley F. Kimball Research Institute, New York Blood Center, New York, New York., Belcher JD; Department of Hematology, Oncology, and Transplantation, University of Minnesota Medical School, Minneapolis, Minnesota., Vercellotti GM; Department of Hematology, Oncology, and Transplantation, University of Minnesota Medical School, Minneapolis, Minnesota., Shi PA; Lindley F. Kimball Research Institute, New York Blood Center, New York, New York.
Jazyk: angličtina
Zdroj: Transfusion [Transfusion] 2018 Feb; Vol. 58 (2), pp. 470-479. Date of Electronic Publication: 2017 Nov 27.
DOI: 10.1111/trf.14407
Abstrakt: 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.
(© 2017 AABB.)
Databáze: MEDLINE
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