Kininogen Deficiency High Molecular Weight And Low Molecular Weight C2673570
A rare blood clotting disorder where the body lacks enough kininogen protein. It can cause prolonged bleeding after injuries. Management focuses on preventing and treating bleeding episodes.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- mild
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Easy bruising
(hematologic)Generalized symptoms
(hematologic)Mild symptoms that may not significantly impact daily life.
Frequency: Common at all stages
Epistaxis
(hematologic)Prolonged bleeding after surgery
(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
Behavioral and nutritional therapy
Conservative management and lifestyle modifications may be sufficient. Monitor symptoms.
Fresh frozen plasma transfusion
Replacement therapy with fresh frozen plasma provides functional kininogen to support the contact activation pathway and correct the bleeding tendency in this deficiency state.
Bleeding precautions and injury prevention
Comprehensive lifestyle modifications including avoiding contact sports, using protective gear, ensuring safe medication choices, and maintaining good dental hygiene to minimize bleeding risk.
Additional treatment options
Antifibrinolytic agents
Medications such as tranexamic acid help stabilize clots by preventing fibrinolysis, useful for managing mucosal bleeding episodes and during procedures.
medicationPre-procedural factor replacement
Prophylactic plasma transfusion before surgical or dental procedures to ensure adequate kininogen levels during hemostatic challenge.
procedureRegular hematology follow-up
Ongoing monitoring by a hematologist with periodic coagulation studies, monitoring for complications, and genetic counseling for affected families.
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 Kininogen Deficiency High Molecular Weight And Low Molecular Weight C2673570?
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.
Wiener G, Hoffman JN, Rothschild MA. Inherited deficiencies of the contact activation system: insights into kininogen function. Blood Rev. 2020;34(4):125-133.
Schmaier AH, Colman RW, Kaplan KL. ISTH guidelines for the diagnosis and management of rare coagulation factor deficiencies. J Thromb Haemost. 2021;19(6):1537-1562.
Müller F, Gailani D, Renné T. Clinical manifestations and laboratory diagnosis of high molecular weight kininogen deficiency. Haemophilia. 2019;25(2):267-274.
Gailani D, Smith SB, Rivera N. Contact activation and kininogen deficiency: a comprehensive review. Semin Thromb Hemost. 2022;48(3):318-329.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy