Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Impaired megakaryocytopoiesis in type 2B von Willebrand disease with severe thrombocytopenia.

Blood - 15 Oct 2006

Nurden Paquita, Debili Najet, Vainchenker William, Bobe Regis, Bredoux Raymonde, Corvazier Elisabeth, Combrie Robert, Fressinaud Edith, Meyer Dominique, Nurden Alan T, Enouf Jocelyne

Abstract excerpt

In type 2B von Willebrand disease, there is spontaneous binding of mutated von Willebrand factor (VWF) multimers to platelets. Here we report a family in which severe thrombocytopenia may also be linked to abnormal megakaryocytopoiesis. A heterozygous mutation in the VWF A1 domain gave a R1308P substitution in an interactive site for glycoprotein Ibalpha (GPIbalpha). Electron microscopy showed clusters of...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Calcium Signaling
Share to Caspases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Megakaryocytes

Share to Platelet Membrane Glycoproteins

Share to Thrombocytopenia

Share to Thrombopoiesis

Share to von Willebrand Diseases

Share to von Willebrand Factor

Source

Identifiers

PMID: 16720832
DOI: 10.1182/blood-2006-03-009449

Genes

VWF
TPO

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Impaired megakaryocytopoiesis in type 2B von Willebrand disease with severe thrombocytopenia.PMID 16720832Read ↗

Update on the causes of platelet disorders and functional consequencesSemantic proximity Read ↗

Platelet-type von Willebrand disease: a rare, often misdiagnosed and underdiagnosed bleeding disorder.Semantic proximity Read ↗

Type 2B von Willebrand Disease: A Matter of Plasma Plus Platelet Abnormality.Semantic proximity Read ↗

A type 2b von Willebrand disease mutation (Ile546-->Val) associated with an unusual phenotype.Semantic proximity Read ↗

Diagnosis of platelet-type von Willebrand disease by flow cytometrySemantic proximity Read ↗

Clinical, laboratory and therapeutic aspects of platelet-type von Willebrand disease.Semantic proximity Read ↗

Type 2B von Willebrand disease associated with the release of platelet agglutinates from megakaryocytes in the bone marrow.Semantic proximity Read ↗

Platelet-type von Willebrand disease: new insights into the molecular pathophysiology of a unique platelet defect.Semantic proximity Read ↗

Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients.Semantic proximity Read ↗

A family having type 2B von Willebrand disease with a novel VWF p.R1308S mutation: Detection of characteristic platelet aggregates on peripheral blood smears as the key aspect of diagnosis.Semantic proximity Read ↗

Type 2M:Milwaukee-1 von Willebrand disease: an in-frame deletion in the Cys509-Cys695 loop of the von Willebrand factor A1 domain causes deficient binding of von Willebrand factor to platelets.Semantic proximity Read ↗

Bernard-Soulier syndrome: an update.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.