Learn About Methemoglobinemia

What is the definition of Methemoglobinemia?

Methemoglobinemia (MetHb) is a blood disorder in which an abnormal amount of methemoglobin is produced. Hemoglobin is the protein in red blood cells (RBCs) that carries and distributes oxygen to the body. Methemoglobin is a form of hemoglobin.

With methemoglobinemia, the hemoglobin can carry oxygen, but is not able to release it effectively to body tissues.

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What are the alternative names for Methemoglobinemia?

Hemoglobin M disease; Erythrocyte reductase deficiency; Generalized reductase deficiency; MetHb

What are the causes of Methemoglobinemia?

The MetHb condition can be:

  • Passed down through families (inherited or congenital)
  • Caused by exposure to certain medicines, chemicals, or foods (acquired)

There are two forms of inherited MetHb. The first form is passed on by both parents. The parents usually do not have the condition themselves. They carry the gene that causes the condition. It occurs when there is a problem with an enzyme called cytochrome b5 reductase.

There are two types of inherited MetHb:

  • Type 1 (also called erythrocyte reductase deficiency) occurs when RBCs lack the enzyme.
  • Type 2 (also called generalized reductase deficiency) occurs when the enzyme doesn't work in the body.

The second form of inherited MetHb is called hemoglobin M disease. It is caused by defects in the hemoglobin protein itself. Only one parent needs to pass on the abnormal gene for the child to inherit the disease.

Acquired MetHb is more common than the inherited forms. It occurs in some people after they are exposed to certain chemicals and medicines, including:

  • Anesthetics such as benzocaine
  • Nitrobenzene
  • Certain antibiotics (including dapsone and chloroquine)
  • Nitrites (used as additives to prevent meat from spoiling)
What are the symptoms of Methemoglobinemia?

Symptoms of type 1 MetHb include:

  • Bluish coloring of the skin (called cyanosis)

Symptoms of type 2 MetHb include:

  • Developmental delay
  • Failure to thrive
  • Intellectual disability
  • Seizures

Symptoms of hemoglobin M disease include:

  • Bluish coloring of the skin

Symptoms of acquired MetHb include:

  • Bluish coloring of the skin
  • Headache
  • Giddiness
  • Altered mental state
  • Fatigue
  • Shortness of breath
  • Lack of energy
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What are the current treatments for Methemoglobinemia?

People with hemoglobin M disease don't have symptoms. So, they may not need treatment.

A medicine called methylene blue is used to treat severe MetHb. Methylene blue may be unsafe in people who have or may be at risk for a blood disease called G6PD deficiency. They should not take this medicine. If you or your child has G6PD deficiency, always tell your provider before getting treatment.

Ascorbic acid may also be used to reduce the level of methemoglobin.

Alternative treatments include hyperbaric oxygen therapy, red blood cell transfusion and exchange transfusions.

In most cases of mild acquired MetHb, no treatment is needed. But you should avoid the medicine or chemical that caused the problem. Severe cases may need a blood transfusion.

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What is the outlook (prognosis) for Methemoglobinemia?

People with type 1 MetHb and hemoglobin M disease often do well. Type 2 MetHb is more serious. It often causes death within the first few years of life.

People with acquired MetHb often do very well once the medicine, food, or chemical that caused the problem is identified and avoided.

What are the possible complications of Methemoglobinemia?

Complications of MetHb include:

  • Shock
  • Seizures
  • Death
When should I contact a medical professional for Methemoglobinemia?

Call your provider if you:

  • Have a family history of MetHb
  • Develop symptoms of this disorder

Call 911 or the local emergency number right away if you have severe shortness of breath.

How do I prevent Methemoglobinemia?

Genetic counseling is suggested for couples with a family history of MetHb and are considering having children.

Babies 6 months or younger are more likely to develop methemoglobinemia. Therefore, homemade baby food purees made from vegetables containing high levels of natural nitrates, such as carrots, beetroots, or spinach should be avoided.

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What are the latest Methemoglobinemia Clinical Trials?
A Phase II Study of Topical Sodium Nitrite in Patients With Sickle Cell and Leg Ulcers

Summary: The investigators propose a Phase II study, prospective and placebo controlled, of a topical cream containing sodium nitrite compared to the current standard of care. Sodium nitrite is a local donor of nitric oxide, which is known to improve blood flow and decrease bacterial load in the ulcer bed. The primary objectives are to evaluate the safety of topical sodium nitrite cream treatment in patien...

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Use of Methylene Blue in Acquired Methemoglobinemia: Prospective Observational Registry (metHb)

Summary: This prospective, observational registry aims to collect real-world data regarding the safety and efficacy of ProvayBlue® (methylene blue 0.5%) used according to normal standard of care for the treatment of acquired methemoglobinemia. Methylene blue has been used for decades as a rescue medication for the treatment of methemoglobinemia, a rare and potentially life-threatening condition in which el...

What are the Latest Advances for Methemoglobinemia?
Dapsone for Refractory Gastrointestinal Symptoms in Children With Immunoglobulin A Vasculitis.
Safety and practicality of high dose inhaled nitric oxide in emergency department COVID-19 patients.
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Methylene blue in management of COVID19.
Who are the sources who wrote this article ?

Published Date: April 29, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Benz EJ, Ebert BL. Hemoglobin variants associated with hemolytic anemia, altered oxygen affinity, and methemoglobinemias. In: Hoffman R, Benz EJ, Silberstein LE, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 43.

Letterio J, Pateva I, Petrosiute A, Ahuja S. Hematologic and oncologic problems in the fetus and neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 79.

Means RT. Approach to the anemias. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 149.