Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Essential athrombia: study of a new case.

S Inceman, Y Tangün

    Thrombosis Et Diathesis Haemorrhagica
    |April 30, 1975
    PubMed
    Summary
    This summary is machine-generated.

    This study reports a rare constitutional platelet function disorder in a young girl, marked by impaired platelet aggregation. This condition, essential athrombia, highlights the critical role of platelet aggregation in hemostasis.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    The frequency of tuberculosis in adult allogeneic stem cell transplant recipients in Turkey.

    Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation·2000
    Same author

    High risk of chronic graft-versus-host disease in unmanipulated allogeneic peripheral blood stem cell transplantation.

    Blood·1998
    Same author

    Cyclosporin-related reversible muscular toxicity.

    Bone marrow transplantation·1998
    Same author

    Evaluation of chimerism with DNA polymorphisms in bone marrow transplantation.

    The Turkish journal of pediatrics·1997
    Same author

    Frequency of factor V Leiden (Arg506Gln) in Turkey.

    British journal of haematology·1997
    Same author

    Frequency of factor V Leiden in Turkey.

    International journal of hematology·1996
    Same journal

    Letter: Fibrinolytic activity in the endometrial adenocarcinoma.

    Thrombosis et diathesis haemorrhagica·1975
    Same journal

    Clinical and genetic aspects of Glanzmann's thrombasthenia in Israel: report of 22 cases.

    Thrombosis et diathesis haemorrhagica·1975
    Same journal

    Evans blue: a specific inhibitor of factor VIII-induced platelet agglutination.

    Thrombosis et diathesis haemorrhagica·1975
    Same journal

    Letter: The buffering of anticoagulants for blood collection.

    Thrombosis et diathesis haemorrhagica·1975
    Same journal

    Letter: Clinical use of prothrombin complex and risk of hepatitis.

    Thrombosis et diathesis haemorrhagica·1975
    Same journal

    Letter: Measurement of mild factor VIII inhibitors in Bethesda units.

    Thrombosis et diathesis haemorrhagica·1975
    See all related articles

    Area of Science:

    • Hematology
    • Platelet Physiology
    • Hemostasis

    Background:

    • Constitutional platelet function disorders are rare and can cause significant bleeding tendencies.
    • Understanding platelet aggregation defects is crucial for diagnosing and managing bleeding disorders.
    • Essential athrombia, first described in the mid-20th century, represents a specific type of platelet aggregation defect.

    Purpose of the Study:

    • To characterize a case of constitutional platelet function disorder in a 12-year-old girl.
    • To investigate the specific platelet aggregation abnormalities and related functional defects.
    • To confirm the existence and features of essential athrombia using modern diagnostic methods.

    Main Methods:

    • Turbidimetric assessment of platelet aggregation in response to various agonists (ADP, epinephrine, thrombin, collagen, Ristocetin).

    Related Experiment Videos

  • Evaluation of platelet factor 3 activity, clot retraction, and bleeding time.
  • Analysis of adenosine diphosphate (ADP) release from platelets stimulated by collagen and thrombin, and kaolin.
  • Measurement of platelet fibrinogen content.

    • Main Results:

    • The patient exhibited a lifelong bleeding tendency with prolonged bleeding time and normal platelet count.
    • Platelet aggregation was absent with standard adenosine diphosphate (ADP) concentrations but showed a minimal response to very high concentrations.
    • Platelets were unresponsive to epinephrine, thrombin, and diluted collagen but responded to strong collagen and Ristocetin.
    • Platelet ADP release was normal with collagen and thrombin but impaired with kaolin; platelet fibrinogen was normal.

    Conclusions:

    • The case confirms a primary functional platelet disorder solely characterized by an aggregation defect, consistent with essential athrombia.
    • This disorder presents with a specific pattern of unresponsiveness to multiple agonists, impacting primary hemostasis.
    • Modern investigation methods validate the distinct nature of this aggregation defect, emphasizing its clinical significance.