Hereditary Spherocytosis Type 5 C2675192
A rare form of hereditary spherocytosis with abnormally shaped red blood cells that are destroyed faster than normal. It causes varying degrees of anemia and may lead to gallbladder problems. Management includes monitoring, medications, and sometimes surgical removal of the spleen.
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)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
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Splenectomy
Surgical removal of the spleen reduces hemolysis and improves anemia in hereditary spherocytosis. Laparoscopic approach is preferred when feasible.
Folate Supplementation
Daily folic acid supplementation supports increased erythropoiesis in patients with chronic hemolysis. Helps prevent folate deficiency anemia.
Additional treatment options
Red Blood Cell Transfusion
Supportive blood transfusion for symptomatic anemia or acute hemolytic crisis. Used when hemoglobin drops significantly below patient's baseline.
procedureRegular Hematologic Monitoring
Periodic blood counts and bilirubin monitoring to assess disease severity and transfusion needs. Helps determine optimal timing for potential splenectomy.
monitoringPre-Splenectomy Vaccinations
Comprehensive vaccination against encapsulated organisms including pneumococcal, meningococcal, and Haemophilus influenzae type b before splenectomy.
medicationMedication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy