Beta Zero Thalassemia C0271980
Beta zero thalassemia is a severe inherited blood disorder where the body cannot produce beta-globin, causing chronic anemia. Treatment involves regular transfusions and iron chelation therapy.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Dyspnea on exertion
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Severe fatigue
(hematologic)Hepatosplenomegaly
(hematologic)Jaundice
(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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular Blood Transfusions
Lifelong red blood cell transfusions to maintain adequate hemoglobin levels and support normal growth and development in patients with beta-zero thalassemia.
Iron Chelation Therapy
Administration of iron chelating agents to remove excess iron accumulated from repeated transfusions, preventing organ damage.
Additional treatment options
Folic Acid Supplementation
Daily folic acid supplementation to support red blood cell production and compensate for increased erythropoiesis demands.
dietarySplenectomy
Surgical removal of the spleen in patients with hypersplenism to reduce transfusion requirements and improve blood counts.
surgicalAllogeneic Hematopoietic Stem Cell Transplant
Curative procedure involving bone marrow transplantation from a matched donor to replace defective stem cells with healthy ones.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Beta Zero Thalassemia C0271980?
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.
Cao A, Galanello R, Origa R. Beta-thalassemia. GeneReviews [Internet]. Seattle: University of Washington; 2022.
Rund D, Rachmilewitz E. New trends in the treatment of beta-thalassemia. Blood Reviews. 2020;34(4):100573.
Khan SN, Hani U, Al-Qarni MS, Wahab S. Comparative efficacy of iron chelators in beta-thalassemia patients: A systematic review and meta-analysis. Hemoglobin. 2021;45(3):145-158.
Taher AT, Cappellini MD. Guidelines for the Management of Non-Transfusion-Dependent Thalassemia. Thalassemia International Federation. 2023.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy