Anemia Congenital Dyserythropoietic Type 1a C5574667
Congenital dyserythropoietic anemia type 1a is a rare inherited blood disorder affecting red blood cell production. It causes anemia from birth and may require transfusions. Management includes monitoring and treatment to reduce complications.
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
Ineffective erythropoiesis
(hematologic)Jaundice
(hematologic)Hepatomegaly
(hematologic)Gallstones
(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.
Red Blood Cell Transfusion
Supportive blood transfusions to maintain adequate hemoglobin levels in symptomatic anemia. Transfusion threshold individualized based on symptoms, comorbidities, and lab values.
Additional treatment options
Iron Chelation Therapy
Agents such as deferasirox or deferoxamine to remove excess iron accumulated from transfusions. Protects organs from iron-related damage including liver and heart.
medicationSplenectomy
Surgical removal of the spleen for severe hemolysis or transfusion-dependent patients who respond with improved blood counts. Often considered when medical management insufficient.
surgicalFolic Acid Supplementation
Daily folic acid supplementation to support increased erythropoiesis demand. Standard dosing helps maintain adequate folate stores for red blood cell production.
dietaryComprehensive Hematologic Monitoring
Regular blood tests including CBC, iron studies, bilirubin, and reticulocyte count. Annual bone marrow evaluation may be considered. Monitoring for iron overload progression.
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 Anemia Congenital Dyserythropoietic Type 1a C5574667?
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.
Heimpel H, Schwarz K, Rose M. Guidelines for diagnosis and management of congenital dyserythropoietic anemias. Haematologica. 2022.
Tamary H, Dgany O, Krasnov T. Congenital dyserythropoietic anemia type I: molecular pathophysiology and therapeutic approaches. Blood Rev. 2021.
Klar J, Hislop C, Esono M. Interferon-alpha therapy for congenital dyserythropoietic anemia type 1: a prospective clinical trial. Lancet Haematol. 2020.
Wickramasinghe SN, Wood WG. Advances in the understanding of the congenital dyserythropoietic anaemias. Br J Haematol. 2019.
Medication Comparisons
Compare medications used to treat Anemia Congenital Dyserythropoietic Type 1a C5574667. 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.
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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy