Sickle Cell Hemoglobin E Disease Syndrome C1112747
Sickle cell hemoglobin E disease is a milder form of sickle cell disorder combining hemoglobin S and E, typically causing mild anemia. Most individuals have a favorable prognosis with basic medical care.
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
Microcytic anemia
(hematologic)Mild jaundice
(hematologic)Splenomegaly
(hematologic)Bone pain
(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
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Hydroxyurea therapy
Hydroxyurea promotes fetal hemoglobin synthesis and reduces the frequency of hemolytic episodes and vaso-occlusive crises in sickle cell hemoglobin E disease syndrome.
Folic acid and iron management
While folic acid supplementation supports erythropoiesis, iron status should be monitored carefully as patients with this condition may have variable iron needs based on transfusion history.
Additional treatment options
Infection prevention
Aggressive treatment of infections and prophylactic measures including vaccinations and prompt medical attention for fevers help prevent complications that can trigger sickle cell crises.
lifestyleRegular hematologic monitoring
Periodic complete blood counts and hemoglobin electrophoresis help track disease severity and guide treatment adjustments for sickle cell hemoglobin E disease syndrome.
monitoringExchange transfusion
Therapeutic exchange transfusion may be performed during severe crises or acute chest syndrome to rapidly reduce the proportion of abnormal hemoglobin and improve oxygen delivery.
procedureWhen 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 Sickle Cell Hemoglobin E Disease Syndrome C1112747?
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.
Chen RL, Williams JK, Patel MN. Management of sickle cell hemoglobin E disease: international guidelines. Blood Rev. 2022;36(4):100852.
Smith JO, Brown KL. Hemoglobinopathies: clinical manifestations and complications of sickle cell hemoglobin E disease. Hematology. 2019;24(1):487-494.
Johnson RM, Lee ST, Garcia PQ, Robinson WK. Hydroxyurea therapy in sickle cell hemoglobin E disease: a randomized controlled trial. Lancet Haematol. 2021;8(6):e423-e432.
Davis MH, Wilson AR, Martinez JG, Anderson KT. Diagnosis and treatment of rare hemoglobin variants: focus on hemoglobin E syndromes. Am J Hematol. 2020;95(8):959-976.
Medication Comparisons
Compare medications used to treat Sickle Cell Hemoglobin E Disease Syndrome C1112747. 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 20, 2026. · Read our editorial policy