ʻO Thrombotic thrombositopenic purpura

ʻO Thrombotic thrombositopenic purpura (TTP) kahi maʻi koko kahi e kū ai nā ʻōiwi platelet i nā kīʻaha koko liʻiliʻi. ʻO kēia ke alakaʻi i kahi helu platelet haʻahaʻa (thrombositopenia).

Hoʻokumu ʻia kēia maʻi e nā pilikia me kahi ʻākena (kahi ʻano protein) i pili i ka hoʻopili ʻia ʻana o ke koko. Kapa ʻia kēia enzyme ADAMTS13. Loaʻa ka loaʻa ʻole o kēia enzyme i ka ʻōpala platelet. ʻO Platelet kahi ʻāpana o ke koko e kōkua i ke kāpili ʻana o ke koko.

I ka hui pū ʻana o nā platelet, ʻoi aku ka liʻiliʻi o nā platelet i loaʻa i ke koko i nā ʻāpana ʻē aʻe o ke kino e kōkua i ke kāpili ʻana. Hiki i kēia ke alakaʻi i ke kahe ʻana ma lalo o ka ʻili.

I kekahi mau hihia, ua hala ka maʻi i waena o nā ʻohana (hoʻoilina). I kēia mau hihia, hānau ʻia ka poʻe me nā pae haʻahaʻa haʻahaʻa o kēia enzyme.

Hoʻokumu ʻia kēia ʻano e:

• Kanesa
• ʻO Chemotherapy
• Ke transplantation o ke koʻokoʻo Hematopoietic
• Maʻi HIV
• ʻO ka Hormone replacement therapy a me nā estrogen
• Nā lāʻau (e laʻa me ticlopidine, clopidogrel, quinine, a me cyclosporine A)

Pākuʻi ʻia paha nā ʻōuli i kekahi o kēia mau mea:

• Kahe i ka ʻili a i ʻole nā ​​membrane mucus
• Huikau
• Ka luhi, nāwaliwali
• Fiva
• ʻEha poʻo
• ʻO kaʻili o kaʻili pale a melemele paha
• Pokole o ka hanu
• Ka helu wikiwiki o ka puʻuwai (ma kahi o 100 mau kani i kēlā me kēia minuke)

ʻO nā hoʻokolohua i kauoha ʻia e komo pū:

• Pākuʻi hana ADAMTS 13
• Bilirubin
• ʻIli koko
• Helu piha koko (CBC)
• Pae Creatinine
• Pae dehydrogenase (LDH) pae
• Heluna pāluna
• Urinalysis
• ʻO Haptoglobin
• Hōʻike Coombs

Loaʻa paha iā ʻoe kahi lāʻau i kapa ʻia ʻo plasma exchange. Lawe ia i kāu plasma maʻamau a hoʻololi iā ia me ka plasma maʻamau mai kahi mea hāʻawi maikaʻi. ʻO ka Plasma ka ʻāpana wai o ke koko i loaʻa nā huna koko a me nā platelet. Hoʻololi pū ka hoʻololi plasma i ka enzyme i nalowale.

Hana ʻia ke ʻano hana penei:

• ʻO ka mea mua, huki ʻia kou koko me he mea lā e hāʻawi i ke koko.
• Ke hele nei ke koko i loko o kahi mīkini e hoʻokaʻawale i ke koko i kāna mau ʻāpana like ʻole, lawe ʻia ka plasma maʻamau a mālama ʻia kāu mau huna koko.
• Hoʻohui ʻia kou mau hunaola koko me ka plasma maʻamau mai kahi mea hāʻawi, a laila hāʻawi ʻia iā ʻoe.

Hoʻomaʻamaʻa mau ʻia kēia hana i kēlā me kēia lā a hōʻike ka hoʻomaikaʻi ʻana i ke koko.

ʻO ka poʻe e pane ʻole i kēia ʻano lāʻau a i ʻole hoʻi e hoʻi pinepine ke ʻano.

• E ʻoki kino e hoʻoneʻe i ko lākou ākea
• E kiʻi i nā lāʻau e kāohi i ka ʻōnaehana pale, e like me nā steroid a i ʻole rituximab

ʻO ka hapanui o ka poʻe i loaʻa i ka pānaʻi pānaʻi e ola hou. Akā make kekahi poʻe i kēia maʻi, ʻoiai inā ʻaʻole ʻike koke ʻia kēia maʻi. I ka poʻe i ola ʻole, hiki i kēia ʻano ke lilo i wā lōʻihi (mau).

Komo pū nā pilikia:

• Hewa ʻole nā ​​hakuʻala
• Helu platelet haʻahaʻa (thrombositopenia)
• Helu koko ʻulaʻula haahaa (hoʻokumu ʻia e ka wā hiapo o nā ʻulaʻula koko)
• Nā pilikia ʻōnaehana hopohopo
• Kahe nui ke koko (hemorrhage)
• Hahau

Kāhea i kāu mea mālama ola inā loaʻa iā ʻoe kahi kahe e wehewehe ʻole ʻia.

Ma muli o ka ʻike ʻole ʻia o ke kumu, ʻaʻohe ala i ʻike ʻia e pale ai i kēia ʻano.

TTP

• Nā pūnaewele koko

ʻO Abrams CS. Thrombositopenia. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 172.

Pūnaewele National Heart, Lung, a me Blood Institute. ʻO Thrombotic thrombositopenic purpura. www.nhlbi.nih.gov/health-topics/thrombotic-thrombositopenic-purpura. Loaʻa iā Malaki 1, 2019.

ʻO Schneidewend R, Epperla N, Friedman KD. Thrombotic thrombositopenic purpura a me ka hemolytic uremic maʻi pale. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 134.