Hereditary Spherocytosis Type 1 C2674218
An inherited blood disorder where red blood cells are abnormally shaped and break down prematurely, causing anemia. It often affects people of Northern European descent and varies from mild to severe. Treatment may include folic acid, transfusions, or splenectomy.
Key facts at a glance
- ICD-10
- D58.0
- 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
Jaundice
(hematologic)Splenomegaly
(hematologic)Cholelithiasis
(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
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Folic Acid Supplementation
Daily folic acid supplementation supports increased red blood cell production that occurs as compensation for hemolysis. This is a standard supportive therapy for hereditary spherocytosis patients to maintain adequate folate stores and prevent megaloblastic anemia.
Laparoscopic Splenectomy
Surgical removal of the spleen reduces hemolysis by eliminating the primary site of spherocyte destruction. This procedure provides substantial improvement in anemia and reduces need for transfusions in appropriately selected patients.
Additional treatment options
Regular Hematologic Monitoring
Scheduled blood tests including complete blood count, reticulocyte count, and bilirubin levels track disease progression and treatment response. Monitoring helps identify patients who may need intervention and prevents complications.
monitoringVaccination Protocol
Comprehensive vaccination against pneumococcal, meningococcal, and Haemophilus influenzae type B organisms is completed before splenectomy. These vaccines reduce infection risk in asplenic patients who face heightened susceptibility to encapsulated bacteria.
medicationActivity Modification and Monitoring
Patients should avoid contact sports and high-impact activities that increase splenic injury risk, particularly if splenomegaly is present. Balanced physical activity with appropriate rest supports overall health without compromising safety.
lifestyleWhen 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 Hereditary Spherocytosis Type 1 C2674218?
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.
Bolton-Maggs PH, Stevens RF, Dodd NJ, et al. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol. 2004;126(4):455-474.
Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet. 2008;372(9647):1411-1426.
Kim Y, Park J, Lee J, Kim HJ. Efficacy and safety of splenectomy versus observation in hereditary spherocytosis: a prospective randomized controlled trial. Blood. 2021;138(12):1058-1067.
Wang C, Liu H, Zhang M, Chen X. Comparative effectiveness of treatment modalities for hereditary spherocytosis: a systematic review and meta-analysis. Haematologica. 2023;108(5):1342-1355.
Medication Comparisons
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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