Glanzmann thrombasthenia

Glanzmann thrombasthenia o se le masani ai le faʻamaʻi o papala toto. Platelet o se vaega o le toto e fesoasoani i le faʻaputuga toto.

Glanzmann thrombasthenia e mafua mai i le le lava o se porotini e masani ona i luga o luga o lapisi. E manaʻomia lenei vailaʻau mo palatete e faʻaputuputu faʻatasi e fai ai faʻaputuga toto.

O le tulaga e fanau mai, o lona uiga na i ai talu mai fanau mai. E tele faʻasologa masani e mafua ai le tulaga.

Faʻailoga mafai ono aofia ai soʻo se tasi o mea nei:

• Toto tele i le taimi ma pe a uma le taotoga
• Pulu toto
• Maua gofie
• Toto mamafa totoga
• Nosebleeds e le faigofie ona taofi
• O le toto faʻaumiumi ma tamaʻi manuʻa

O faʻataʻitaʻiga nei e ono faʻaaoga e faʻailoaina ai lenei tulaga:

• Faitau atoa le toto (CBC)
• Lafoaʻiga o faʻatasiga faʻapitoa
• Lafoaʻiga o auiliiliga galuega (PFA)
• Prothrombin taimi (PT) ma taimi thromboplastin taimi (PTT)

O isi suʻega atonu e manaʻomia. Atonu e manaʻomia foʻi ona faʻataʻitaʻia tagata o le aiga.

E leai se faʻapitoa togafitiga mo lenei faʻamaʻi. O faʻamaʻa totoga e mafai ona faia i tagata o loʻo tele le toto.

O faʻalapotopotoga nei o loʻo avea ma punaoa lelei mo faʻamatalaga i luga o le Glanzmann thrombasthenia:

• Genetic and Rare Diseases Information Center (GARD) - rarediseases.info.nih.gov/diseases/2478/glanzmann-thrombasthenia
• National Organisation for Rare Disorder (NORD) - rarediseases.org/rare-diseases/glanzmann-thrombasthenia

Glanzmann thrombasthenia o se olaga atoa, ma e leai se fofo. E tatau ona e uia ni laʻasaga faʻapitoa e taumafai e aloese mai le palapala pe a maua oe i lenei faʻamaʻi.

Soʻo se tasi e maua i le faʻamaʻi toto e tatau ona alofia le inuina o aspirin ma isi vailaʻau e le faʻaaogaina o le anti-inflammatory (NSAIDs) pei o ibuprofen ma naproxen. O nei vailaʻau mafai faʻafualoa taimi tafetotoi ala i le puipuia peleti mai faʻapipiʻi.

Faʻafitauli e ono aofia ai:

• Toto tele
• Uʻamea le lava o le anemia i taimi faʻafuaseʻi fafine ona o le le masani ai mamafa mamafa

Valaʻau lau fomaʻi pe a fai:

• Oe maua i le toto poʻo le maamaa o se le iloa mafuaʻaga
• Ole toto e le taofia pe a uma togafitiga masani

Glanzmann thrombasthenia o se tofi tuufaasolo. E leai se iloa puipuia.

Faamaʻi o Glanzmann; Thrombasthenia - Glanzmann

Bhatt MD, Ho K, Chan AKC. Faʻaletonu o le coagulation i le neonate. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Faavae Autu ma Faʻataʻitaʻiga. 7th ed. Filatelefia, PA: Elsevier; 2018: mata 150

Nichols WL. Von Willebrand faamaʻi ma le hemorrhagic abnormalities o platelet ma gaioiga gaioiga. I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 25th lomiga. Filatelefaia, PA: Elsevier Saunders; 2016: mata 173