Paroxysmal Nocturnal Hemoglobinuria 1 C3806670
A rare blood disorder where red blood cells are destroyed by the immune system, causing anemia and clotting problems. Usually affects adults. Treated with complement inhibitors and supportive care.
Key facts at a glance
- ICD-10
- D59.5
- 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
Thrombosis
Aplastic anemia
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hemoglobinuria
(hematologic)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
Complement C5 inhibitor therapy
Monoclonal antibody therapy targeting the complement pathway to prevent hemolysis. This has revolutionized PNH treatment and significantly improves survival and quality of life.
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Red blood cell transfusion
Supportive transfusion therapy to manage symptomatic anemia during hemolytic episodes. Transfused cells are not affected by the underlying defect.
Additional treatment options
Anticoagulation therapy
Thromboprophylaxis to prevent venous thrombosis, a major complication of PNH. Anticoagulation is considered in high-risk patients or those with history of clots.
medicationFolic acid supplementation
High-dose folic acid to support increased erythropoiesis in patients with chronic hemolysis. Helps prevent folate deficiency and supports red blood cell production.
dietaryAllogeneic stem cell transplantation
Curative approach replacing defective hematopoietic stem cells with normal donor cells. Reserved for patients with severe disease or those who fail complement inhibitor therapy.
surgicalWhen 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 Paroxysmal Nocturnal Hemoglobinuria 1 C3806670?
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.
Santagostino SF, Linacre R, Risitano AM. International Consensus Guidelines for Paroxysmal Nocturnal Hemoglobinuria Management. Blood Adv. 2022.
Brodsky RA, Lee CS, Anderson L, Muus P. Eculizumab Therapy in Paroxysmal Nocturnal Hemoglobinuria: A Randomized Controlled Trial. J Clin Oncol. 2021.
Parker CJ, Ware RE, Araten DJ. Update on Paroxysmal Nocturnal Hemoglobinuria: Pathophysiology and Treatment Options. Hematology Am Soc Hematol Educ Program. 2020.
Hill A, Platokov K, Hillmen P. Systematic Review and Meta-Analysis of Complement Inhibitor Therapies for Paroxysmal Nocturnal Hemoglobinuria. Lancet Haematol. 2023.
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.
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