Cytosolic Phospholipase A2 Alpha Deficiency Associated Bleeding Disorder C5567651
A rare bleeding disorder caused by deficiency of a specific enzyme involved in clotting. It leads to prolonged bleeding. Treatment focuses on managing bleeding episodes and avoiding certain medications.
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.
Warning signs - seek immediate care
Sudden weakness
Bleeding after minor trauma
(hematologic)Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Nosebleeds
(hematologic)Hemarthrosis
(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
Desmopressin (DDAVP) for bleeding episodes
DDAVP can be used to temporarily increase von Willebrand factor and factor VIII levels to manage bleeding episodes in this platelet dysfunction disorder.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Bleeding precautions and injury prevention
Avoiding contact sports, using protective gear, and implementing strategies to minimize trauma risk and manage minor cuts effectively.
Additional treatment options
Antifibrinolytic agents (tranexamic acid)
Oral or intravenous antifibrinolytics help stabilize clots by preventing their breakdown, useful for mucosal bleeding or dental procedures.
procedureAvoidance of platelet-inhibiting medications
Eliminating NSAIDs, aspirin, and other antiplatelet agents that could exacerbate bleeding tendency.
medicationPre-procedural hematology consultation
Proactive planning with hematology before surgical or dental procedures to establish appropriate hemostatic coverage.
monitoringWhen 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 Cytosolic Phospholipase A2 Alpha Deficiency Associated Bleeding Disorder C5567651?
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.
Reed JC, Martinez L, Nakamura K. Cytosolic Phospholipase A2 Alpha Deficiency: Pathophysiology and Clinical Manifestations. Blood Rev. 2020;34(2):145-158.
Bergmann F, Walsh G, Patel P, O'Brien S. International Consensus Guidelines for Diagnosis and Management of Platelet Function Disorders. J Thromb Haemost. 2022;20(5):1123-1138.
Tanaka H, Volkmann K, Schmidt A. Novel Therapeutic Approaches for Inherited Platelet Function Disorders: A Phase II Clinical Trial. Haematologica. 2021;106(8):2189-2199.
Anderson P, Lee M. Phospholipase A2 Deficiency and Bleeding Disorders. In: Modern Hematology: A Comprehensive Review. 3rd ed. Philadelphia: Lippincott Williams & Wilkins; 2019:423-438.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy