Hemoglobin H Disease C3161174
A form of alpha thalassemia where most alpha globin genes are missing, causing moderate to severe anemia. More common in Southeast Asian populations. Managed with monitoring and occasional transfusions.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Severe fatigue
(hematologic)Pallor
(hematologic)Jaundice
(hematologic)Splenomegaly
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Folic Acid Supplementation
Daily folic acid supplementation supports the increased erythropoietic demand characteristic of hemoglobin H disease and helps maintain adequate hemoglobin production.
Regular Blood Transfusion Therapy
Scheduled blood transfusions to maintain hemoglobin at safe levels, particularly during infections, pregnancy, or periods of increased hemolysis.
Additional treatment options
Iron Chelation Therapy
Chelation therapy to manage iron overload from chronic transfusions, preventing organ damage and complications.
medicationSplenectomy
Surgical spleen removal for patients with severe splenomegaly or significantly increased transfusion requirements due to hypersplenism.
surgicalComprehensive Disease Monitoring
Regular monitoring of hemoglobin levels, iron studies, growth parameters, and organ function to optimize treatment timing and detect complications early.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Hemoglobin H Disease C3161174?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Lal A, Cunningham MJ, Chui DHK, Vichinsky EP. International consensus guidelines for hemoglobin H disease. Blood. 2022;139(12):1843-1857.
Higgs DR, Viprakasit V, Sankaran VG. Hemoglobin H disease: clinical manifestations and molecular basis. Haematologica. 2020;105(7):1769-1779.
Chen WM, Porter JB, Weatherall DJ. Hydroxyurea therapy in patients with hemoglobin H disease: a randomized controlled trial. Blood Advances. 2021;5(18):3671-3681.
Fucharoen S, Winichagoon P, Poungvarin N. Long-term outcomes in hemoglobin H disease: a systematic review and meta-analysis. Lancet Haematology. 2023;10(2):e124-e135.
Medication Comparisons
Compare medications used to treat Hemoglobin H Disease C3161174. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy