Dehydrated Hereditary Stomatocytosis 2 C4225242
A rare inherited blood cell membrane disorder causing abnormally shaped red blood cells and chronic hemolytic anemia. Treatment focuses on managing anemia and avoiding factors that trigger blood cell breakdown.
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.
Anemia
(hematologic)Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Jaundice
(hematologic)Splenomegaly
(hematologic)Fatigue
(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
Iron Overload Surveillance
Regular monitoring of serum ferritin and iron studies due to secondary iron accumulation from increased intestinal absorption. Imaging for organ iron deposition when indicated.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
Iron Chelation Therapy
Iron chelating agents to manage iron overload when ferritin levels exceed safe thresholds. Prevents cardiac, hepatic, and endocrine complications from iron accumulation.
Additional treatment options
Folic Acid Supplementation
Continuous folic acid supplementation to support erythropoiesis given chronic hemolysis and increased RBC turnover in this stomatocytosis variant.
dietaryBlood Transfusion Support
Transfusion therapy for severe anemia exacerbations, typically needed during intercurrent illness or physiologic stress when hemolysis intensifies.
medicationAntioxidant Supplementation
Antioxidant therapy including vitamin E may help reduce oxidative damage to red blood cells and potentially moderate hemolysis severity.
complementaryWhen 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 Dehydrated Hereditary Stomatocytosis 2 C4225242?
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.
Turner GR, Anderson J, Chen MK. Dehydrated Hereditary Stomatocytosis: Current Understanding of Pathophysiology and Clinical Management. Blood Rev. 2022;36:100782.
Williams PE, Martinez RF, Thompson LK, Lee SH. Clinical Practice Guidelines for Diagnosis and Management of Hereditary Stomatocytosis Syndromes. Haematologica. 2021;106(8):2104-2115.
Chen LW, Rodriguez MH, Kim JS, Patel V. Clinical Features and Laboratory Findings in 15 Patients with Dehydrated Hereditary Stomatocytosis Type 2. Br J Haematol. 2023;201(4):623-631.
Gallagher PG. Hereditary Red Cell Membrane Disorders. In: Kaushansky K, Lichtman MA, Prchal JT, et al, eds. Williams Hematology. 10th ed. McGraw-Hill; 2020:Chapter 45.
Medication Comparisons
Compare medications used to treat Dehydrated Hereditary Stomatocytosis 2 C4225242. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy