TY - JOUR
T1 - Recommendations for diagnosis and treatment of methemoglobinemia
AU - SWG of red cell and iron of EHA and EuroBloodNet
AU - Iolascon, Achille
AU - Bianchi, Paola
AU - Andolfo, Immacolata
AU - Russo, Roberta
AU - Barcellini, Wilma
AU - Fermo, Elisa
AU - Toldi, Gergely
AU - Ghirardello, Stefano
AU - Rees, Davis
AU - Van Wijk, Richard
AU - Kattamis, Antonis
AU - Gallagher, Patrick G.
AU - Roy, Noemi
AU - Taher, Ali
AU - Mohty, Razan
AU - Kulozik, Andreas
AU - De Franceschi, Lucia
AU - Gambale, Antonella
AU - De Montalembert, Mariane
AU - Forni, Gian Luca
AU - Harteveld, Cornelis L.
AU - Prchal, Josef
N1 - Publisher Copyright:
© 2021 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
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U2 - 10.1002/ajh.26340
DO - 10.1002/ajh.26340
M3 - Article
C2 - 34467556
AN - SCOPUS:85115639423
VL - 96
SP - 1666
EP - 1678
JO - American Journal of Hematology
JF - American Journal of Hematology
SN - 0361-8609
IS - 12
ER -