Hemoglobin H Disease Nondeletional C3279561
Alpha thalassemia caused by mutations rather than deletions, leading to abnormal alpha chains and anemia. Can be more severe than deletional forms. Treatment depends on symptom severity.
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.
Fatigue
(hematologic)Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
High-Dose Folic Acid Supplementation
Higher-dose folic acid supplementation to support the markedly increased erythropoiesis characteristic of nondeletional hemoglobin H disease, which tends to be more severe.
Regular Transfusion Program
More frequent blood transfusions typically required for nondeletional hemoglobin H disease compared to deletional forms due to more severe hemolysis and anemia.
Additional treatment options
Iron Chelation Therapy
Early and aggressive iron chelation therapy is often necessary due to the combination of transfusion-related iron overload and increased intestinal iron absorption.
medicationGenetic Counseling
Genetic counseling for patients and family members to understand inheritance patterns, carrier status implications, and family planning options.
complementaryIntensive Disease Monitoring
Close monitoring including frequent hemoglobin checks, iron studies every 3 months, cardiac and hepatic MRI for iron overload assessment, and growth monitoring in children.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
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Frequently asked questions
Common questions about this topic.
What is Hemoglobin H Disease Nondeletional C3279561?
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.
Taher AT, Weatherall DJ, Cappellini MD. Guidelines for the management of non-transfusion dependent thalassemia. Blood. 2021;137(17):2347-2356.
Piel FB, Weatherall DJ. The alpha-thalassemias. N Engl J Med. 2020;383(10):961-972.
Viprakasit V, Ekwattanakit S, Chinda K. Clinical phenotype and genotype of nondeletional hemoglobin H disease: a prospective cohort study. Br J Haematol. 2019;186(3):478-486.
Origa R, Danjou F, Cossa S. Hemoglobin H disease: from diagnosis to genetic counseling. Hematology Am Soc Hematol Educ Program. 2022;2022(1):478-485.
Medication Comparisons
Compare medications used to treat Hemoglobin H Disease Nondeletional C3279561. 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