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Glanzmann thrombasthenia

ORPHA:849DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (2)

ITGA2B
integrin subunit alpha 2b
Disease-causing germline mutation(s) in
OMIM: 607759
ITGB3
integrin subunit beta 3
Disease-causing germline mutation(s) in
OMIM: 173470

Фенотипы (20)

Очень частый (80–99%)2
HP:0003010Prolonged bleeding time
HP:0004406Spontaneous, recurrent epistaxis
Частый (30–79%)4
HP:0000225Gingival bleeding
HP:0000978Bruising susceptibility
HP:0004846Prolonged bleeding after surgery
HP:0030137Prolonged bleeding following circumcision
Периодический (5–29%)7
HP:0000132Menorrhagia
HP:0000979Purpura
HP:0002239Gastrointestinal hemorrhage
HP:0007420Spontaneous hematomas
HP:0012587Macroscopic hematuria
HP:0031364Ecchymosis
HP:0400008Menometrorrhagia
Очень редкий (1–4%)1
HP:0011871Impaired ristocetin-induced platelet aggregation
Исключён (0%)6
HP:0004866Impaired ADP-induced platelet aggregation
HP:0008148Impaired epinephrine-induced platelet aggregation
HP:0011870Impaired arachidonic acid-induced platelet aggregation
HP:0011872Impaired thrombin-induced platelet aggregation
HP:0011894Impaired thromboxane A2 agonist-induced platelet aggregation
HP:0031128Impaired collagen-related peptide-induced platelet aggregation

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы