Thrombotic Thrombositopenic Purpura: He aha ia, kumu a me ka mālama ʻana

Anter

• Nā kumu o PTT
• Nā hōʻailona nui a me nā ʻōuli
• Pehea e hana ʻia ai ke kuhi
• Hana Hana PTT

ʻO Thrombotic thrombositopenic purpura, a i ʻole PTT, kahi maʻi hematological kakaikahi akā hiki ke make i ʻike ʻia e ka hoʻokumu ʻia ʻana o ka thrombi liʻiliʻi i loko o nā kīʻaha koko a ʻoi aku ka maʻamau o ka poʻe ma waena o 20 a 40 mau makahiki.

I ka PTT aia ka hoʻēmi nui ʻia o ka helu o nā platelet, i ka hoʻohui ʻana i ka fiva a, i ka hapanui o nā hihia, hōʻino neurological ma muli o ka loli o ke kahe o ke koko i ka lolo ma muli o nā clots.

ʻO ka hōʻailona o PTT i hana ʻia e ka hematologist a i ʻole ka mea hana maʻamau e like me nā ʻōuli a me ka hopena o ka helu piha o ke koko a me ka hamo ʻana o ke koko a pono e hoʻomaka koke ʻia ka hoʻomaʻamaʻa ma hope, no ka mea he maʻi ka maʻi ma kahi o 95% ke mālama ʻole ʻia.

Nā kumu o PTT

ʻO Thrombotic thrombositopenic purpura ka mea nui i hoʻokumu ʻia e ka hemahema a i ʻole ka loli ʻana o ka ʻākena, ADAMTS 13, ʻo ia ke kuleana no ka hana ʻana i nā mole o von Willebrand mea liʻiliʻi, a makemake i kā lākou hana. Aia ka von Willebrand factor i nā platelet a nona ke kuleana no ka paepae ʻana i ka hoʻopili platelet i ka endothelium, e hoʻemi ana a me ka hoʻōki ʻana i ke kahe ʻana.

No laila, i ka loaʻa ʻole o ka ADAMTS 13 enzyme, noho mau nā molekona von Willebrand a ua hoʻopilikia ʻia ke kaʻina stagnation o ke koko a aia kahi manawaʻoi aku ka nui o ka hoʻokumu ʻōpala.

No laila, he mau kumu hoʻoilina ko ka PTT, i kūlike i ka hemahema o ADAMTS 13, a i loaʻa paha, ʻo ia nā mea e alakaʻi i ka hōʻemi ʻana i ka helu o nā platelet, e like me ka hoʻohana ʻana i nā lāʻau immunosuppressive a chemotherapeutic a i ʻole nā ​​agena antiplatelet, nā maʻi, nā hemahema hānai a i ʻole. nā maʻi autoimmune, no ka laʻana.

Nā hōʻailona nui a me nā ʻōuli

Hōʻike pinepine ʻo PTT i nā ʻōuli kikoʻī ʻole, eia naʻe he mea maʻamau ia no nā mea maʻi me ka PTT i manaʻo ʻia he 3 paha o kēia mau ʻano aʻe:

1. Kaha thrombocythemia;
2. Hemolytic anemia, ʻoiai ka thrombi i hana ai i makemake i ka lysis o nā ʻulaʻula koko;
3. Fiva;
4. Thrombosis, hiki ke hana i loko o nā kino o ke kino;
5. ʻEha nui o ka ʻōpū ma muli o ka ischemia ʻōpū;
6. Ka hōʻino ʻana o ke kōpaʻa;
7. Ka hoʻohaunaele Neurological, i hiki ke ʻike ʻia ma o ka hōʻeha poʻo ʻana, ka huikau o ka noʻonoʻo, ka hiamoe a me ke koma pū kekahi.

He mea maʻamau ia no nā mea maʻi me ka PTT i manaʻo ʻia e loaʻa nā ʻōuli o ka thrombositopenia, e like me ke ʻano o ka poni a ʻulaʻula paha ma ka ʻili, kaomi ʻana i nā gum a i ʻole ma o ka ihu, me ke kaohi paʻakikī i ke kahe ʻana o ke koko mai nā ʻeha liʻiliʻi. E ʻike i nā ʻōuli ʻē aʻe o ka thrombositopenia.

ʻO nā disfunction a me nā neurological nā pilikia nui o PTT a kū aʻe i ka wā e keʻakeʻa ai ka thrombi liʻiliʻi i ke ala o ke koko i nā puʻupaʻa ʻelua a me ka lolo, kahi e hopena ai i ka hōʻino a me ka hahau ʻana, no ka laʻana. I mea e hōʻalo ai i nā pilikia, he mea nui ia ke hiki mai nā hōʻailona mua, nīnau ʻia kahi loio a hematologist paha i hiki ke hoʻomaka ʻia ka ʻike a me ka lāʻau.

Pehea e hana ʻia ai ke kuhi

Hana ʻia ke kuhi o ka thrombotic thrombositopenic purpura ma muli o nā ʻōuli i hōʻike ʻia e ke kanaka, me ka hopena o ka helu o ke koko, kahi e ʻike ʻia ai ka emi ʻana o ka nui o nā platelet, i kapa ʻia ʻo thrombositopenia, me ka nānā pū ʻana i ka ʻo ka hoʻopili ʻana o ka platelet i ka platelet, ʻo ia ka manawa e paʻa pū ai nā platelet, me ka schizocytes, nā ʻāpana o nā hunaola ʻulaʻula, no ka mea, hele nā ​​hunaolaʻulaʻula i nā kīʻaha koko i ālai ʻia e nā moku liʻiliʻi.

Hiki ke kauoha ʻia kekahi mau hoʻokolohua ʻē aʻe e kōkua i ke kuhi o PTT, e like me ka wā kahe o ke kahe, i hoʻonui ʻia, a me ka ʻole a i ʻole ka hōʻemi ʻana o ka enzyme ADAMTS 13, ʻo ia kekahi o nā kumu o ka hoʻokumu ʻia o ka thrombi liʻiliʻi.

Hana Hana PTT

Pono e hoʻomaka i ka hoʻomaʻamaʻa no ka thrombotic thrombositopenic purpura i ka wā hiki loa, no ka mea he make ia i ka hapa nui o nā hihia, ʻoiai hiki i ka thrombi ke hana i nā aʻa i hiki i ka lolo, e hoʻemi ana i ke kahe o ke koko i kēlā wahi.

ʻO ka mālama maʻamau i hōʻike ʻia e ka hematologist he plasmapheresis, kahi kaʻina hana kānana koko kahi e ʻoi aku ai ka nui o nā mea ʻawelike i kēia maʻi a me ka mea nui o von Willebrand factor, me ka mālama ʻana i nā kōkua kākoʻo, e like me hemodialysis, no ka laʻana. , inā loaʻa ka hōʻino hōʻino. Hoʻomaopopo pehea ka plasmapheresis e hana ai.

Eia kekahi, ʻo ka hoʻohana ʻana i nā corticosteroids a me nā lāʻau immunosuppressive, no ka laʻana, e koi ʻia e ke kauka, i mea e hakakā ai i ke kumu o ka PTT a pale i nā pilikia.