Recommendations for diagnosis and treatment of methemoglobinemia

SWG of red cell and iron of EHA and EuroBloodNet, Achille Iolascon, Paola Bianchi, Immacolata Andolfo, Roberta Russo, Wilma Barcellini, Elisa Fermo, Gergely Toldi, Stefano Ghirardello, Davis Rees, Richard Van Wijk, Antonis Kattamis, Patrick G. Gallagher, Noemi Roy, Ali Taher, Razan Mohty, Andreas Kulozik, Lucia De Franceschi, Antonella Gambale, Mariane De MontalembertGian Luca Forni, Cornelis L. Harteveld, Josef Prchal

Research output: Contribution to journalArticlepeer-review

Abstract

Methemoglobinemia is a rare disorder associated with oxidization of divalent ferro-iron of hemoglobin (Hb) to ferri-iron of methemoglobin (MetHb). Methemoglobinemia can result from either inherited or acquired processes. Acquired forms are the most common, mainly due to the exposure to substances that cause oxidation of the Hb both directly or indirectly. Inherited forms are due either to autosomal recessive variants in the CYB5R3 gene or to autosomal dominant variants in the globin genes, collectively known as HbM disease. Our recommendations are based on a systematic literature search. A series of questions regarding the key signs and symptoms, the methods for diagnosis, the clinical management in neonatal/childhood/adulthood period, and the therapeutic approach of methemoglobinemia were formulated and the relative recommendations were produced. An agreement was obtained using a Delphi-like approach and the experts panel reached a final consensus >75% of agreement for all the questions.

Original languageEnglish
Pages (from-to)1666-1678
JournalAmerican Journal of Hematology
Volume96
Issue number12
DOIs
Publication statusPublished - 2021

ASJC Scopus subject areas

  • Hematology

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