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Essential thrombocythemia

ORPHA:3318DiseaseMultigenic/multifactorial, Not applicableAll ages

Ассоциированные гены (6)

TET2
tet methylcytosine dioxygenase 2
Biomarker tested in
OMIM: 612839
JAK2
Janus kinase 2
Disease-causing somatic mutation(s) in
OMIM: 147796
MPL
MPL proto-oncogene, thrombopoietin receptor
Disease-causing somatic mutation(s) in
OMIM: 159530
CALR
calreticulin
Disease-causing somatic mutation(s) in
OMIM: 109091
SH2B3
SH2B adaptor protein 3
Disease-causing somatic mutation(s) in
OMIM: 605093
TP53
tumor protein p53
Biomarker tested in
OMIM: 191170

Фенотипы (30)

Очень частый (80–99%)15
HP:0001658Myocardial infarction
HP:0001872Abnormality of thrombocytes
HP:0001894Thrombocytosis
HP:0003010Prolonged bleeding time
HP:0003401Paresthesia
HP:0004420Arterial thrombosis
HP:0004936Venous thrombosis
HP:0005513Increased megakaryocyte count
HP:0005561Abnormality of bone marrow cell morphology
HP:0011875Abnormal platelet morphology
HP:0012378Fatigue
HP:0031388Megakaryocyte nucleus hyperlobulation
HP:0100576Amaurosis fugax
HP:0100659Abnormality of the cerebral vasculature
HP:0100749Chest pain
Частый (30–79%)6
HP:0001892Abnormal bleeding
HP:0002076Migraine
HP:0001744Splenomegaly
HP:0002315Headache
HP:0002321Vertigo
HP:0100785Insomnia
Периодический (5–29%)9
HP:0000505Visual impairment
HP:0000978Bruising susceptibility
HP:0001974Leukocytosis
HP:0002326Transient ischemic attack
HP:0002488Acute leukemia
HP:0002863Myelodysplasia
HP:0011974Myelofibrosis
HP:0030243Hepatic vein thrombosis
HP:0032147Erythromelalgia

Эпидемиология (5)

Point prevalence
1-5 / 10 000
United States
Annual incidence
1-9 / 100 000
Sweden
Point prevalence
1-5 / 10 000
Sweden
Point prevalence
1-5 / 10 000
Europe
Annual incidence
1-9 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы