Enfermedad de Von Willebrand: Tipo III, una revisión de la literatura

Palabras clave: enfermedad de von Willebrand, factor de von Willebrand, hemorragia

Resumen

La Enfermedad de Von Willebrand es característica por sus manifestaciones clínicas provocadas por la alteración de la cantidad o de la morfología estructural del factor de Von Willebrand, en el tipo 3 existe una ausencia cuantitativa casi total del FvW, el objetivo del presente trabajo es realizar una búsqueda bibliográfica acerca de la fisiopatología, genética, cuadro clínico, diagnóstico y manejo terapéutico en el segundo nivel de atención; realizado en base a información científica encontrada en artículos, guías actualizadas, documentos físicos y virtuales que tengan información sobre la enfermedad de Von Willebrand utilizando diferentes bases de datos tales como: UptoDate, Elsevier, Pubmed, ProQuest, Stat Pearls, National Library of medicine, Scielo, Stat Pearls, Google Académico, empleando para la búsqueda: "Von Willebrand Diseases", "Von Willebrand Diseases/classification", "Von Willebrand-Diseases/complications", "Von Willebrand Diseases/epidemiology" "Enfermedad de Von Willebrand" publicados hace 3-5 años en Inglés y español. La EvW tipo 3 es la manifestación más grave que se expresa fenotípicamente en hemorragias de mucosas, tejidos blandos, articulaciones y los músculos. para su diagnóstico además de la clínica se necesitan exámenes de sangre en donde se evidenciará trombocitopenia, anemia microcítica, tiempo de tromboplastina parcial prolongado, para el tratamiento, se realiza manejo sintomático con concentrados de FVW y factor VIII.

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Citas

Baronciani, Luciano, and Flora Peyvandi. 2020. “How We Make an Accurate Diagnosis of von Willebrand Disease.” Thrombosis Research 196(June):579–89. doi: 10.1016/j.thromres.2019.07.010.

Canadian Hemophilia Society. 2018. Guidelines for the Diagnosis and Management of von Willebrand Disease (VWD).

Castillo, R. 2020. “Enfermedad de Von Willebrand Novedades En Diagnóstico de Laboratorio.” Revista Bioanalisis 111(16):616–18. doi: 10.15517/rc_ucr-hsjd.v3i10.12811.

Chapin, John. 2018. “Von Willebrand Disease in the Elderly: Clinical Perspectives.” Clinical Interventions in Aging 13:1531–41. doi: 10.2147/CIA.S136931.

Denis, Cécile V., Sophie Susen, and Peter J. Lenting. 2021. “Von Willebrand Disease: What Does the Future Hold?” Blood 137(17):2299–2306. doi: 10.1182/blood.2020008501.

Itzhar-Baikian, Nathalie, Pierre Boisseau, Bérangère Joly, and Agnès Veyradier. 2019. “Updated Overview on von Willebrand Disease: Focus on the Interest of Genotyping.” Expert Review of Hematology 12(12):1023–36. doi: 10.1080/17474086.2019.1670638.

James, Paula. 2022a. “Acquired von Willebrand Syndrome (AvWS).” Vnitrni Lekarstvi 68(4):E16–19. doi: 10.36290/vnl.2022.055.

James, Paula. 2022b. “Von Willebrand Disease (VWD): Treatment of Minor Bleeding and Routine Care.” UpToDate 1–38.

James, Paula D., Nathan T. Connell, Barbara Ameer, Jorge Di Paola, Jeroen Eikenboom, Nicolas Giraud, Sandra Haberichter, Vicki Jacobs-Pratt, Barbara Konkle, Claire McLintock, Simon McRae, Robert R. Montgomery, James S. O’Donnell, Nikole Scappe, Robert Sidonio, Veronica H. Flood, Nedaa Husainat, Mohamad A. Kalot, and Reem A. Mustafa. 2021. “ASH ISTH NHF WFH 2021 Guidelines on the Diagnosis of von Willebrand Disease.” Blood Advances 5(1):280–300. doi: 10.1182/BLOODADVANCES.2020003265.

Kalot, Mohamad A., Nedaa Husainat, Abdallah El Alayli, Omar Abughanimeh, Osama Diab, Sammy Tayiem, Bader Madoukh, Ahmad B. Dimassi, Aref Qureini, Barbara Ameer, Jeroen C. J. Eikenboom, Nicolas Giraud, Claire McLintock, Simon McRae, Robert R. Montgomery, James S. O’Donnell, Nikole Scappe, Robert F. Sidonio, Romina Brignardello-Petersen, Veronica H. Flood, Nathan T. Connell, Paula D. James, and Reem A. Mustafa. 2022. “Von Willebrand Factor Levels in the Diagnosis of von Willebrand Disease: A Systematic Review and Meta-Analysis.” Blood Advances 6(1):62–71. doi: 10.1182/bloodadvances.2021005430.

Leebeek, F. W. G., and S. Susen. 2018. “Von Willebrand Disease: Clinical Conundrums.” Haemophilia 24(April):37–43. doi: 10.1111/hae.13508.

Ma, Alice. 2022. “Approach to the Adult with a Suspected Bleeding Disorder.” UpToDate 1–32. Retrieved February 7, 2023 (https://www.uptodate.com/contents/approach-to-the-adult-with-a-suspected-bleeding-disorder).

Mannucci, Pier Mannuccio. 2019. “New Therapies for von Willebrand Disease.” Hematology (United States) 2019(1):590–95. doi: 10.1182/hematology.2019000368.

Miesbach, Wolfgang. 2020. “Perioperative Management for Patients with von Willebrand Disease: Defining the Optimal Approach.” European Journal of Haematology 105(4):365–77. doi: 10.1111/ejh.13462.

Miesbach, Wolfgang, and Erik Berntorp. 2021. “Translating the Success of Prophylaxis in Haemophilia to von Willebrand Disease.” Thrombosis Research 199(January):67–74. doi: 10.1016/j.thromres.2020.12.030.

Ng, Christopher J., and Jorge Di Paola. 2018. “Von Willebrand Disease: Diagnostic Strategies and Treatment Options.” Pediatric Clinics of North America 65(3):527–41. doi: 10.1016/j.pcl.2018.02.004.

Nguyen, Trinh, and Lakshmi V. Srivaths. 2020. “Von Willebrand Disease.” Hematology in the Adolescent Female 29–42. doi: 10.1007/978-3-030-48446-0_3.

O’Donnell, James S., and Ross I. Baker. 2023. “Low von Willebrand Disease: A Bleeding Disorder of Unknown Cause?” Hamostaseologie 43(1):44–51. doi: 10.1055/a-1980-8198.

O’Sullivan, Jamie M., Ellia Tootoonchian, Baiba Ziemele, Michael Makris, Augusto B. Federici, Claudia Khayat Djambas, Magdy El Ekiaby, Dawn Rotellini, Robert F. Sidonio, Alfonso Iorio, Donna Coffin, Glenn F. Pierce, Jeffrey Stonebraker, Paula D. James, and Michelle Lavin. 2023. “Von Willebrand Disease: Gaining a Global Perspective.” Haemophilia (February):1104–12. doi: 10.1111/hae.14804.

Palinkas, L., S. Horwitz, and C. Green. 2021. “New Developments in von Willebran Disease.” Physiology & Behavior 176(1):139–48. doi: 10.1111/bjh.16681.New.

Paula James. 2023a. “Pathophysiology of von Willebrand Disease.” UpToDate 1–38. Retrieved February 7, 2023 (https://www.uptodate.com/contents/pathophysiology-of-von-willebrand-disease?search=hemofilia tratamiento&topicRef=1307&source=see_link).

Paula James. 2023b. “Von Willebrand Disease (VWD): Treatment of Major Bleeding and Major Surgery.” Uptodate 1–23. Retrieved February 7, 2023 (https://www.uptodate.com/contents/von-willebrand-disease-vwd-treatment-of-major-bleeding-and-major-surgery?search=von willebrand &source=search_result&selectedTitle=3~150&usage_type=default&display_rank=3).

Rao, Emily Southard, and Christopher J. Ng. 2018. “Current Approaches to Diagnostic Testing in von Willebrand Disease.” Transfusion and Apheresis Science 57(4):463–65. doi: 10.1016/j.transci.2018.07.005.

Rick, Margaret E., Lawrence Leung, and Stephen Landaw. 2023. “Clinical Presentation and Diagnosis of von Willebrand Disease.Pdf.” Up To DateUp To Date.

Sabih, Ayan, and Hani Babiker. 2018. “Von Willebrand Disease.” Consults in Obstetric Anesthesiology 663–65. doi: 10.1007/978-3-319-59680-8_179.

Smock, Kristi J. 2023. “Von Willebrand Factor Testing Ratios in the Diagnosis and Subtyping of von Willebrand Disease.” International Journal of Laboratory Hematology 45(S2):23–29. doi: 10.1111/ijlh.14074.

Tebo, Collin, Colin Gibson, and Maryann Mazer-Amirshahi. 2020. “Hemophilia and von Willebrand Disease: A Review of Emergency Department Management.” Journal of Emergency Medicine 58(5):756–66. doi: 10.1016/j.jemermed.2020.02.019.

Valentino, Leonard A., Peter L. Turecek, Herbert Gritsch, Saulius Butenas, and Kenneth G. Mann. 2018. “Issues Complicating Precision Dosing for Factor VIII Prophylaxis.” Transfusion and Apheresis Science 57(4):472–79. doi: 10.1016/j.transci.2018.07.007.

Zolkova, Jana, Juraj Sokol, Tomas Simurda, Lubica Vadelova, Zuzana Snahnicanova, Dusan Loderer, Miroslava Dobrotova, Jela Ivankova, Ingrid Skornova, Zora Lasabova, Peter Kubisz, and Jan Stasko. 2020. “Genetic Background of von Willebrand Disease: History, Current State, and Future Perspectives.” Seminars in Thrombosis and Hemostasis 46(4):484–500. doi: 10.1055/s-0039-3402430.

Publicado
2023-08-16
Cómo citar
Martinez Carrera , C. A., & Cruz Castillo, Y. M. (2023). Enfermedad de Von Willebrand: Tipo III, una revisión de la literatura. Ciencia Latina Revista Científica Multidisciplinar, 7(4), 3746-3769. https://doi.org/10.37811/cl_rcm.v7i4.7220
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Artículos