Transfusion Therapy - Special Considerations
Hemophilia
Hemophilia A and B are rare, sex-linked diseases affecting males almost exclusively. Hemophilia A is due to an abnormality in factor VIII, while hemophilia B (Christmas disease) is due to a factor IX abnormality. The incidence in the United States is 1:10,000 males for hemophilia A and 1:100,000 males for hemophilia.
- Clinical features. Patients usually present early in childhood with hemarthroses and soft tissue hematomas after minor trauma. Laboratory tests demonstrate a markedly prolonged PTT with normal PT and platelet count.
- Treatment with the appropriate factor (of either recombinant or lyophilized concentrate source) should be coordinated with the patient's hematologist. Hemophilia A is treated with factor VIII to achieve preoperative activity levels of 25% to 100%, depending on the extent of the procedure. Some patients with mild hemophilia A may respond to DDAVP therapy. In an emergency, if factor VIII is unavailable, cryoprecipitate transfusion can provide the deficient factor. Hemophilia B is treated with factor IX to achieve at least 30% to 50% activity before surgery.
There's more to see -- the rest of this topic is available only to subscribers.
Citation
Pino, Richard M., editor. "Transfusion Therapy - Special Considerations." Clinical Anesthesia Procedures, 9th ed., Wolters Kluwer, 2019. Anesthesia Central, anesth.unboundmedicine.com/anesthesia/view/ClinicalAnesthesiaProcedures/728386/all/Transfusion_Therapy___Special_Considerations.
Transfusion Therapy - Special Considerations. In: Pino RMR, ed. Clinical Anesthesia Procedures. Wolters Kluwer; 2019. https://anesth.unboundmedicine.com/anesthesia/view/ClinicalAnesthesiaProcedures/728386/all/Transfusion_Therapy___Special_Considerations. Accessed December 11, 2024.
Transfusion Therapy - Special Considerations. (2019). In Pino, R. M. (Ed.), Clinical Anesthesia Procedures (9th ed.). Wolters Kluwer. https://anesth.unboundmedicine.com/anesthesia/view/ClinicalAnesthesiaProcedures/728386/all/Transfusion_Therapy___Special_Considerations
Transfusion Therapy - Special Considerations [Internet]. In: Pino RMR, editors. Clinical Anesthesia Procedures. Wolters Kluwer; 2019. [cited 2024 December 11]. Available from: https://anesth.unboundmedicine.com/anesthesia/view/ClinicalAnesthesiaProcedures/728386/all/Transfusion_Therapy___Special_Considerations.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC
T1 - Transfusion Therapy - Special Considerations
ID - 728386
ED - Pino,Richard M,
BT - Clinical Anesthesia Procedures
UR - https://anesth.unboundmedicine.com/anesthesia/view/ClinicalAnesthesiaProcedures/728386/all/Transfusion_Therapy___Special_Considerations
PB - Wolters Kluwer
ET - 9
DB - Anesthesia Central
DP - Unbound Medicine
ER -