Platelet-type pseudovonoid hemophilia

Introduction to platelet-type pseudovascular hemophilia Platelet-type pseudovascular hemophilia, characterized by different hemorrhagic symptoms, reduced plasma high molecular weight vWF multimer, due to some characteristics of vascular hemophilia (vWD), but its defect lies in GPIb /IX, therefore, the disease is also known as pseudo-vWD or platelet-type vWD. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: nosebleeds

The cause of platelet-type pseudovascular hemophilia

Genetic factors (85%):

The disease is autosomal dominant, and its basic defect is the molecular abnormality of GPIb. Because platelets are combined with high molecular weight vWF multimers, plasma vWF is reduced, and high molecular weight vWF multimers are reduced, and thrombocytopenia is The clarification of the GPIb sequence has revealed two mutations in three families: Gly233Val and Met239Val.

Other factors (15%):

A genetic mutation caused by environmental factors or a specific substance during embryonic development.

Pathogenesis

However, the pathogenesis of this disease has not been fully elucidated.

Platelet-type pseudovascular hemophilia prevention

Pay attention to the usual habits and find timely treatment in time.

Platelet-type pseudovascular hemophilia complications Complications, nosebleeds

Platelet-type pseudovascular hemophilia mild to moderate skin mucosal hemorrhage, such as bleeding gums, nose bleeding, bleeding after tooth extraction, not only after tonsillectomy or other surgical bleeding. However, some patients have no bleeding after surgery.

Symptoms of platelet-type pseudovascular hemophilia Common symptoms Skin mucosal bleeding injection needle eye and surgery... Bleeding after tooth extraction is more than bleeding gums

The main manifestations of this disease are mild to moderate skin mucosal hemorrhage, such as bleeding gums, nose bleeding, bleeding after tooth extraction, bleeding after tonsillectomy or other surgical procedures, bleeding after taking aspirin or other antiplatelet drugs, but Some patients have no bleeding even after surgery.

Examination of platelet-type pseudovascular hemophilia

1. Peripheral blood platelet count decreased, the lowest can be reduced to 20 × 10 ⁹ / L, platelet volume can be increased.

2. The bleeding time is prolonged.

3. Plasma vWF levels are slightly decreased, high molecular weight vWF multimers are absent; ADP, adrenaline, collagen and arachidonic acid-induced platelet aggregation are normal or slightly reduced, and low concentrations of ristocetin-induced platelet aggregation The reaction (RIPA) is increased.

Diagnosis and identification of platelet-type pseudovascular hemophilia

The diagnosis of this disease depends on clinical manifestations and laboratory tests.

Mainly based on the doctor's instructions for early detection and early detection.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.