Pono thrombocythemia

Pono ka thrombositosis (ET) kahi kūlana kahi e hana ai ka iwi iwi i nā pā nui he nui. ʻO Platelet kahi ʻāpana o ke koko e kōkua i ke kāpili ʻana o ke koko.

Nā hualoaʻa ET mai ka hoʻonui ʻana i ka platelet. Ma muli o ka hana ʻole o kēia mau platelet, ʻo nā kahe koko a me ke kahe ʻana he pilikia maʻamau. Hoʻomaʻamaʻa ʻole ʻia, ET e māhuahua i ka hala ʻana o ka manawa.

ʻO ET kahi ʻāpana o kahi hui i ʻike ʻia he maʻi myeloproliferative. ʻO nā poʻe ʻē aʻe:

• Leukemia myelogenous maʻi (ka maʻi ʻaʻai e hoʻomaka i ka iwi iwi)
• ʻO Polycythemia vera (ka maʻi o ka iwi iwi e alakaʻi aku ai i kahi piʻi maʻamau o ka helu o nā hunaola koko)
• Myelofibrosis kumu (maʻi o ka iwi iwi kahi i kuapo ʻia ka momona e nāʻiʻo maka fibrous)

Nui ka poʻe me ET i ka hoʻololi o kahi ʻāpana (JAK2, CALR, a i ʻole MPL).

ʻO ET ka mea maʻamau i ka poʻe waena. Hiki ke ʻike ʻia i ka poʻe ʻōpio, ʻo ka wahine hoʻi ma lalo o 40 mau makahiki.

Hiki i nā ʻōuli o ke koko ke komo i kekahi o kēia mau mea:

• He ʻeha poʻo (ʻo ka mea maʻamau)
• Tingling, anuanu, a i ʻole blueness i nā lima a me nā wāwae
• Huʻihuʻi a uila paha
• Nā pilikia ʻike
• Pākuʻi liʻiliʻi (hoʻouka ischemic kuewa) a i ʻole hahau

Inā pilikia ka kahe ʻana i ke koko, hiki i nā ʻōuli ke hoʻopili i kekahi o kēia mau mea:

• Māmā a me nā ihu ihu
• Kahe mai ka gastrointestinal tract, respiratory system, urinary tract, a i ʻole ʻili
• Kahe ʻana mai o ka gum
• ʻO ke kahe lōʻihi ʻana mai ke kaʻina hana a me ka hemo ʻana o ka niho

ʻO ka hapa nui o ka manawa, ʻike ʻia ʻo ET ma o nā hoʻokolohua koko i hana ʻia no nā pilikia olakino ʻē aʻe ma mua o ka hōʻike ʻia ʻana o nā hōʻailona.

Hoʻomaopopo paha kāu mea mālama olakino i kahi ake nui a i ʻole spleen ma ka hoʻokolokolo kino. Hiki paha iā ʻoe ke kahe o ke koko i nā manamana wāwae a i ʻole nā ​​wāwae e hōʻeha i ka ʻili i kēia mau wahi.

Hiki i nā hoʻokolohua ʻē aʻe ke:

• Biopsy iwi ʻōiwi
• Helu piha koko (CBC)
• Nā hoʻāʻo genetic (e nānā i kahi loli o ka JAK2, CALR, a i ʻole ka MPL gene)
• ʻIli acid uric

Inā loaʻa iā ʻoe nā pilikia hoʻoweliweli ola, hiki iā ʻoe ke mālama i kapa ʻia ʻo platelet pheresis. Hoʻoiho koke ia i nā platelet i loko o ke koko.

ʻO ka wā lōʻihi, hoʻohana ʻia nā lāʻau e hoʻemi i ka helu platelet e hōʻalo ai i nā pilikia. ʻO nā lāʻau lapaʻau maʻamau e hoʻohana ʻia me hydroxyurea, interferon-alpha, a anagrelide paha. I kekahi poʻe me ka hoʻololi JAK2, hiki ke hoʻohana ʻia i nā mea kūpikipiki kikoʻī o ka protein JAK2.

I ka poʻe e makaʻu nui ana i ka hoʻopili ʻana, aspirin ma kahi haʻahaʻa haʻahaʻa (81 a 100 mg i kēlā me kēia lā) e hoʻoliʻiliʻi i nā hanana

ʻAʻole pono ka poʻe he nui i kahi lapaʻau, akā pono lākou e ukali pono ʻia e kā lākou mea hāʻawi.

E loli paha nā hopena. Hiki i ka hapa nui o ka poʻe ke hele no nā wā lōʻihi me ka ʻole o nā pilikia a loaʻa ke ola maʻamau. I kahi poʻe liʻiliʻi, hiki i nā pilikia mai ke kahe ʻana o ke koko a me ke kahe o ke koko ke hana i nā pilikia koʻikoʻi.

I nā hihia maʻamau, hiki i ka maʻi ke loli i ka leukemia ākea a i ʻole myelofibrosis.

Komo pū nā pilikia:

• ʻO ka leukemia ākea a i ʻole myelofibrosis
• Kahe nui ke koko (hemorrhage)
• ʻO ka hahau, hōʻeha puʻuwai, a i ʻole ke koko i nā lima a me nā wāwae

Kāhea i kāu mea hāʻawi inā:

• Loaʻa iā ʻoe ke kahe koko ʻole i hoʻomau ʻia ʻoi aku ka lōʻihi ma mua o ka mea pono.
• ʻIke ʻoe i ka ʻeha o ka umauma, ʻeha o ka wāwae, huikau, nāwaliwali, numbness, a i ʻole nā ​​hōʻailona hou aʻe.

ʻO ka thrombocythemia mua; Pono thrombositosis

• Nā pūnaewele koko

Mascarenhas J, Iancu-Rubin C, Kremyanskaya M, Najfeld V, Hoffman R. Pono thrombositosis. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 69.

ʻO Tefferi A. Polycythemia vera, thrombositosis pono, a me myelofibrosis kumu. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 166.