Autosomal Recessive Secondary Polycythemia Not Associated With Vhl Von Hippel Lindau Gene C5191076
A hereditary condition where the body produces too many red blood cells, distinct from VHL-related causes. It follows an autosomal recessive inheritance pattern. Management typically includes phlebotomy to reduce blood thickness and prevent complications.
Key facts at a glance
- ICD-10
- D75.1
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Deep vein thrombosis
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Headache
(hematologic)Dizziness
(hematologic)Facial plethora
(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
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Therapeutic phlebotomy
Regular blood removal to reduce red blood cell mass and lower blood viscosity, decreasing the risk of thrombotic complications associated with polycythemia.
Increased hydration and physical activity
Maintaining adequate fluid intake and regular exercise helps optimize blood viscosity and promotes healthy circulation, supporting overall cardiovascular function.
Additional treatment options
Antiplatelet therapy
Low-dose aspirin may be prescribed to reduce platelet aggregation and decrease thrombotic risk, particularly in patients with elevated cardiovascular risk factors.
medicationRegular hematologic monitoring
Serial measurement of hemoglobin, hematocrit, and red blood cell indices to track disease progression and guide treatment intensity adjustments.
monitoringIron restriction
Dietary modifications to limit iron intake may be recommended in some cases to avoid exacerbating erythrocytosis, though this must be balanced with overall nutritional needs.
dietaryWhen 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 Autosomal Recessive Secondary Polycythemia Not Associated With Vhl Von Hippel Lindau Gene C5191076?
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.
Lee J, Smith KR, Brown MP. Genetic determinants of erythrocytosis and secondary polycythemia. Blood Rev. 2022;45(3):100712.
McMullin MF, Cario H, Johansson P, et al. Guidelines for the diagnosis, investigation and management of polycythemia/erythrocytosis. Br J Haematol. 2023;200(4):423-438.
Patel M, Fernandez CJ, Anand KP. Autosomal recessive familial erythrocytosis: clinical features and genetic basis. Hematology. 2021;26(1):284-291.
Girardot M, Vittet D, Soularue E, et al. Molecular mechanisms underlying familial erythrocytosis and secondary polycythemia. Nat Rev Nephrol. 2020;16(10):573-588.
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