Recommendations for diagnosis and treatment of methemoglobinemia.

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.

• Authors: 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 Montalembert, Gian Luca Forni, Cornelis L Harteveld, Josef Prchal, SWG of red cell and iron of EHA and EuroBloodNet
• Journal: American journal of hematology (Am J Hematol) Vol. 96 Issue 12 Pg. 1666-1678 (12 01 2021) ISSN: 1096-8652 [Electronic] United States
• PMID: 34467556 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: © 2021 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.

Topics

• Consensus
• Diagnosis, Differential
• Disease Management
• Humans
• Methemoglobinemia (diagnosis, physiopathology, therapy)