Acquired methemoglobinemia

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Overview

Acquired methemoglobinemia is a condition where an abnormally high amount of methemoglobin builds up in the blood. Methemoglobin is a form of hemoglobin that cannot carry oxygen effectively. Unlike inherited forms of methemoglobinemia, the acquired type is not caused by a genetic defect you are born with. Instead, it is triggered by exposure to certain drugs, chemicals, or toxins. Common culprits include certain local anesthetics (like benzocaine and lidocaine), nitrates, nitrites, dapsone, and some industrial chemicals. When methemoglobin levels rise too high, the body's tissues do not get enough oxygen, which can become a medical emergency. The hallmark symptom is a bluish or grayish discoloration of the skin, lips, and nail beds, known as cyanosis, that does not improve with supplemental oxygen. Other symptoms include headache, fatigue, dizziness, shortness of breath, confusion, and in severe cases, seizures, coma, or even death. The severity of symptoms depends on how high the methemoglobin level climbs. Mild cases (methemoglobin levels around 10-20%) may cause only slight skin color changes and mild symptoms, while levels above 50% can be life-threatening. The good news is that acquired methemoglobinemia is usually treatable and often reversible once the offending agent is identified and removed. The primary treatment is methylene blue, given intravenously, which works quickly to convert methemoglobin back to normal hemoglobin. In most cases, patients recover fully with prompt treatment. Supportive care, including supplemental oxygen, is also provided. For patients who cannot receive methylene blue (such as those with G6PD deficiency), alternative treatments like ascorbic acid or exchange transfusion may be considered.

Also known as:

Key symptoms:

Bluish or grayish skin color (cyanosis)Blue or gray lips and nail bedsHeadacheFatigue and weaknessDizziness or lightheadednessShortness of breathRapid heart rateConfusion or altered mental statusNauseaChocolate-brown colored bloodAnxiety or irritabilityLoss of consciousness in severe casesSeizures in severe cases

Clinical phenotype terms (21)— hover any for plain English
MethemoglobinemiaHP:0012119AcidosisHP:0001941Loss of consciousnessHP:0007185
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Apr 2016

ProvayBlue: FDA approved

For treatment of pediatric and adult patients with acquired methemoglobinemia.

FDAcompleted

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

ProvayBlue

methylene blue0.5%· Provepharm SAS■ Boxed WarningOrphan Drug

For treatment of pediatric and adult patients with acquired methemoglobinemia.

No actively recruiting trials found for Acquired methemoglobinemia at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Acquired methemoglobinemia community →

Specialists

21 foundView all specialists →
MT
Minh-Ha Tran
PITTSBURGH, PA
Specialist
1 Acquired methemoglobinemia publication
RB
Rabia Basri
JACKSONVILLE, FL
Specialist
1 Acquired methemoglobinemia publication
PD
Pankaj Das
Specialist
1 Acquired methemoglobinemia publication
GS
Gautam Singh
DETROIT, MI
Specialist
1 Acquired methemoglobinemia publication
NK
Nasim Khajavirad
Specialist
1 Acquired methemoglobinemia publication
GD
Ghazal Daftari
Specialist
1 Acquired methemoglobinemia publication
MJ
Mehrasa Raisi Jelodar
Specialist
1 Acquired methemoglobinemia publication
NT
Nichole Tackett
Specialist
1 Acquired methemoglobinemia publication
UR
Umberto Raucci
Specialist
1 Acquired methemoglobinemia publication
HG
Haijuan Gao
ORANGE, CA
Specialist
1 Acquired methemoglobinemia publication
CM
Charles V Pollack, MD
ROSWELL, GA
Specialist
PI on 2 active trials
BM
Bruno Megarbane
Specialist
PI on 1 active trial1 Acquired methemoglobinemia publication
CM
Caleb Mentzer
BOISE, ID
Specialist
1 Acquired methemoglobinemia publication
CM
Charles Morrow
Specialist
1 Acquired methemoglobinemia publication
BT
Brian Thurston
SPARTANBURG, SC
Specialist
1 Acquired methemoglobinemia publication
KL
Kristine Lombardozzi
SPARTANBURG, SC
Specialist
1 Acquired methemoglobinemia publication
TM
Trey Mathews
SPARTANBURG, SC
Specialist
1 Acquired methemoglobinemia publication
MF
Muthanna Thiab Fendi
Specialist
2 Acquired methemoglobinemia publications
ZH
Zainab Ali Hussein
Specialist
2 Acquired methemoglobinemia publications
AB
Ashraf Fhed Mohammed Basalilah
Specialist
2 Acquired methemoglobinemia publications
RA
Rusul Mahdi Abid
Specialist
2 Acquired methemoglobinemia publications

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Acquired methemoglobinemia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Acquired methemoglobinemia

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What specific drug or chemical caused my methemoglobinemia?,Are there other medications or substances I need to avoid in the future?,Should I be tested for G6PD deficiency or any underlying conditions that could make me more vulnerable?,What should I do if I notice symptoms returning?,Should I wear a medical alert bracelet or carry an information card?,Are my family members at any increased risk for this condition?,How can I make sure my other doctors and dentists know about this condition?

Common questions about Acquired methemoglobinemia

What is Acquired methemoglobinemia?

Acquired methemoglobinemia is a condition where an abnormally high amount of methemoglobin builds up in the blood. Methemoglobin is a form of hemoglobin that cannot carry oxygen effectively. Unlike inherited forms of methemoglobinemia, the acquired type is not caused by a genetic defect you are born with. Instead, it is triggered by exposure to certain drugs, chemicals, or toxins. Common culprits include certain local anesthetics (like benzocaine and lidocaine), nitrates, nitrites, dapsone, and some industrial chemicals. When methemoglobin levels rise too high, the body's tissues do not get en

How is Acquired methemoglobinemia inherited?

Acquired methemoglobinemia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Acquired methemoglobinemia?

21 specialists and care centers treating Acquired methemoglobinemia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Acquired methemoglobinemia?

1 patient support program are currently tracked on UniteRare for Acquired methemoglobinemia. See the treatments and support programs sections for copay assistance, eligibility, and contact details.