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November 1, 1977Blood

Molecular defect in platelets from patients with bernard-soulier syndrome

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Key result

An IgG antibody from a Bernard-Soulier syndrome patient induced agglutination in normal platelets but not in platelets from other patients, identifying a missing 150,000 MW membrane antigen.

Population

Platelets from 50 normal subjects and 3 patients with Bernard-Soulier syndrome, and serum from 1 patient…

Design

Preclinical

Authors

LDL DegosGTG TobelemPLP Lethielleux

Discussion

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Overview

May guide antibody-based diagnostics in Bernard-Soulier syndrome; hypothesis-generating and requires prospective human validation.

Structured PICO

P
Population
In vitro laboratory study using serum from 1 patient with Bernard-Soulier syndrome tested against platelets from 50 normal subjects and 3 other patients with the syndrome.
E
Exposure
IgG antibody isolated from the serum of a patient with Bernard-Soulier syndrome
O
Outcome
Platelet agglutination and identification of the antigenic determinantsurrogate

Identified a missing 150,000 MW membrane molecule in platelets of patients with Bernard-Soulier syndrome using a specific IgG antibody.

Cite This Study

Degos et al. (1977) studied Bernard-Soulier syndrome (n=54). IgG antibody from Bernard-Soulier syndrome patient serum vs. Normal platelets vs Bernard-Soulier syndrome platelets was evaluated on Platelet agglutination and identification of missing membrane antigen. An IgG antibody from a Bernard-Soulier syndrome patient induced agglutination in normal platelets but not in platelets from other patients, identifying a missing 150,000 MW membrane antigen.

synapsesocial.com/papers/6aa02424ea9f4728adef7bbehttps://doi.org/10.1182/blood.v50.5.899.bloodjournal505899
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Bernard-Soulier Syndrome: Diagnosis by an ELISA Method Using Monoclonal Antibodies in 2 New Unrelated Patients2009 · 15 citations
  2. 2Additional glycoprotein defects in Bernard-Soulier's syndrome: confirmation of genetic basis by parental analysis1983 · 16 citations
  3. 3Additional glycoprotein defects in Bernard-Soulier's syndrome: confirmation of genetic basis by parental analysis1983 · 180 citations
  4. 4Residual amounts of glycoprotein Ib concomitant with near-absence of glycoprotein IX in platelets of Bernard-Soulier patients1988 · 29 citations
  5. 5Defective adhesion of blood platelets to vascular microfibrils in the Bernard-Soulier syndrome1993 · 8 citations