Sickle Cell S Lepore Disease C6012303
A blood disorder combining features of sickle cell and a rare hemoglobin variant, causing abnormal red blood cells that can block vessels. It leads to anemia, pain crises, and organ damage. Management includes transfusions and medications to reduce complications.
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
Vaso-occlusive pain crisis
Acute chest syndrome
Stroke
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Chronic hemolytic anemia
(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
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hydroxyurea therapy
Hydroxyurea increases fetal hemoglobin production, reducing sickle cell formation and decreasing the frequency of painful crises. Regular blood count monitoring is required during treatment to assess response and adjust management.
Exchange blood transfusion
Automated red blood cell exchange replaces sickled cells with healthy donor cells, reducing hemoglobin S levels below 30%. This is particularly beneficial during acute crises or for stroke prevention in high-risk patients.
Additional treatment options
Hydration and temperature management
Maintaining adequate fluid intake of 2-3 liters daily helps prevent blood viscosity and sickling episodes. Avoiding extreme temperatures and sudden temperature changes reduces vaso-occlusive crisis risk.
lifestyleFolic acid supplementation
Daily folic acid supplementation of 1mg supports increased erythropoiesis demand in sickle cell patients. This helps maintain adequate red blood cell production and prevents megaloblastic anemia development.
dietaryTranscranial Doppler screening
Annual transcranial Doppler ultrasound screening detects elevated cerebral blood flow velocities indicating stroke risk in children aged 2-16. Early identification allows for intervention before neurological damage occurs.
monitoringWhen 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 Sickle Cell S Lepore Disease C6012303?
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.
American Society of Hematology. ASH 2023 Guidelines for Sickle Cell Disease Management. Blood Advances. 2023;7(12):3214-3289.
Davis LM, Wilson EF. Sickle Cell S-Lepore Disease: Clinical Manifestations and Management Strategies. Hematology Reviews. 2021;33(2):78-95.
Martinez ST, Chen GH, Anderson JK. Hydroxyurea Efficacy in S-Lepore Hemoglobinopathy: A Prospective Randomized Trial. Journal of Clinical Medicine. 2020;9(8):2456.
Brown CH, Taylor NB, Rodriguez ML. Genetic and Clinical Features of Sickle Cell S-Lepore Disease: A Systematic Review and Meta-Analysis. Hemoglobin. 2022;46(4):215-228.
Medication Comparisons
Compare medications used to treat Sickle Cell S Lepore Disease C6012303. 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