He aha ka Evans Syndrome? E kamaʻilio kākou e pili ana i ia mea!

Ke luhi nei ʻoe i kekahi manawa? A i ʻole he paʻakikī iā ʻoe ke hoʻōki i ke kahe ʻana o ke koko mai nā mea liʻiliʻi loa? Malia paha no ka mea ke hana kūʻē nei ka ʻōnaehana pale o kou kino iā ʻoe. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi kakaʻikahi akā koʻikoʻi loa e ʻike ai. ʻO ia ka Evans Syndrome . Mai hopohopo, e kamaʻilio mākou e pili ana i nā mea āpau ma nā ʻōlelo maʻalahi.

No laila, he aha ka Evans Syndrome?

I ka ʻōlelo maʻalahi, he maʻi autoimmune ka Evans Syndrome. ʻO ka mea e hana ʻia ana, ʻo nā koa i manaʻo ʻia e pale i ko mākou kino, ʻo ka ʻōnaehana pale, e hoʻomaka hewa ana e luku i kekahi o kā mākou mau hunaola koko ponoʻī. Ua like ia me ko mākou pūʻali koa ponoʻī e hoʻouka ana i ko mākou poʻe ponoʻī. ʻO ka hopena, ua nalowale ko mākou kino i ka nui o nā hunaola koko e pono ai. ʻO kēia emi ʻana o nā hunaola koko ka mea a mākou e kapa nei he cytopenia i ka lāʻau lapaʻau.

ʻO ka hapa nui o ka poʻe me Evans Syndrome ka mea mua e hoʻopilikia ʻia e nā hunaola koko ʻulaʻula haʻahaʻa (kahi maʻi i kapa ʻia ʻo anemia ) a me nā platelets (kahi maʻi i kapa ʻia ʻo thrombocytopenia ). Ma waho aʻe o nā mea ʻelua, hiki i kekahi poʻe ke loaʻa i nā helu haʻahaʻa o kahi ʻano hunaola koko keʻokeʻo i kapa ʻia ʻo neutrophils (kahi maʻi i kapa ʻia ʻo neutropenia ).

Ke haʻahaʻa kēia mau ʻano hunaola koko, nui nā mea nui i loko o ko kākou kino, e like me ka loaʻa ʻana o ka oxygen, ke kāpili ʻana o ke koko, a me ke kaua ʻana i nā maʻi, e hoʻopilikia ʻia. ʻO ia ke kumu e nāwaliwali ai kākou, luhi, hoʻomaka e kahe koko maʻalahi, a maʻi pinepine .

Hiki i kēia maʻi ke hoʻopilikia iki i kekahi poʻe, aʻo kekahi poʻe hiki ke koʻikoʻi loa. ʻAʻole like kona mau hopena i ke ola holoʻokoʻa. ʻO ka mea maikaʻi loa, ʻoiai ʻaʻohe lāʻau lapaʻau no ia mea, hiki i ka lāʻau lapaʻau ke kōkua i ka kaohi ʻana i nā hōʻailona a hoʻēmi i nā hopena .

He aha nā hōʻailona o ka Evans Syndrome?

Hiki ke ʻokoʻa loa nā hōʻailona o ka Evans Syndrome. Aia ia i ke ʻano o ka cytopenia āu e loaʻa ai a me kona koʻikoʻi. I ka manawa pinepine, hiki mua ka anemia a i ʻole ka thrombocytopenia, a ʻike ʻia nā mea ʻē aʻe ma hope.

Nā hōʻailona mua o ka nele o nā ʻula koko ʻulaʻula ( anemia ):

Ua luhi anei ʻoe ma hope o ka hele wāwae ʻana no kahi mamao pōkole? Ke luhi nei ʻoe a ʻaʻole hiki ke hana i nā mea āu i hana ai ma mua? Malia paha ke nānā aku nei kou helehelena i kahi keʻokeʻo iki. Eia kekahi o nā hōʻailona mua o ka anemia :

• Luhi loa ke manaʻo nei.
• ʻIli keʻokeʻo (keʻokeʻo) .
• Māmā ke poʻo a i ʻole ka poniuniu .
• Ka paʻakikī o ka hanu ʻana (dyspnea) .
• Ka wikiwiki o ka puʻuwai (tachycardia) .
• Ke manaʻo nei a i ʻole ka manaʻo e like me ke kuʻi ʻano ʻē ʻana o kou puʻuwai (heart palpitations) .

Nā hōʻailona mua o ka nele o ka platelet (thrombocytopenia):

Ua ʻike anei ʻoe i kekahi manawa e ʻike ʻia nā kiko ʻulaʻula liʻiliʻi ma kou ʻili? A i ʻole loaʻa iā ʻoe nā palapū ma kou kino holoʻokoʻa? Manaʻo anei ʻoe e kahe ana ke koko o kou mau ʻāʻī ma mua o ka maʻamau ke palaki ʻoe i kou mau niho? Eia nā hōʻailona hiki ke hōʻike ʻia he haʻahaʻa nā platelets:

• Nā kiko kahe koko ʻulaʻula liʻiliʻi (petechiae) ma lalo o ka ʻili.
• Nā kiko poni ma ka ʻili (purpura) .
• ʻO ka palapū (ecchymosis) i kū mai me ke kumu ʻole.
• Ke kahe nui ʻana o ke koko mai nā ʻāʻī a i ʻole ka ihu.
• Ke kahe koko nui i ka wā o ka menstruation i nā wahine.
• Ke kahe ʻana o ke koko me ka ʻōpala a i ʻole ka mimi.

Nā hōʻailona o ka neutropenia (hiki paha kēia mau mea a ʻaʻole paha e hana ma hope):

He mea maʻamau ka loaʻa ʻana o ke anu i hoʻokahi a ʻelua paha i ka mahina. Akā, maʻi pinepine ʻoe me ke kuni? Loaʻa mau iā ʻoe nā ʻeha waha? Me he mea lā e lōʻihi ka manawa e ho'ōla ai? Eia kekahi mau mea e hiki ke hana ke haʻahaʻa kāu neutrophils:

• Kuni.
• Nā ʻeha waha .
• Anu pinepine, kuni, a me ka ʻeha o ka ʻōpū.
• Nā maʻi pepeiao e hoʻi hou ana.
• Nā maʻi fungal no ka wā lōʻihi.
• Nā maʻi ʻōpū mimi (UTI) .

ʻO nā hōʻailona ʻē aʻe o ka Evans Syndrome (hiki ke loaʻa kēia mau mea i kekahi manawa):

• Nā ʻōpuʻu lymph pehu i ka ʻāʻī.
• ʻŌpū nui .
• Ake i hoʻonui ʻia .
• ʻŌpū ʻino .

No ke aha e kū mai ai ka Evans Syndrome?

E like me kā mākou i ʻōlelo ai ma mua, he maʻi autoimmune ka Evans Syndrome. ʻO ke ʻano kēia, ua kuhihewa ka ʻōnaehana pale o ko mākou kino i kekahi o ko mākou mau hunaola koko ponoʻī he "ʻenemi" a hoʻomaka e hoʻouka iā lākou.

Hana ʻia kēia hana e nā pūnaewele B, kahi ʻano pūnaewele kūikawā i loko o kā mākou ʻōnaehana pale. Hana kēia mau pūnaewele B i kahi mea i kapa ʻia he antibodies . Hele kēia mau antibodies a hopu i kēlā mau pūnaewele koko hala ʻole a luku iā lākou. ʻO ka maʻamau, hoʻomaka lākou e hoʻouka i hoʻokahi ʻano pūnaewele koko mua, a laila hiki iā lākou ke hoʻomaka e hoʻouka i nā ʻano pūnaewele koko ʻē aʻe.

ʻO kēia kaʻina hana ke kumu hope loa e loaʻa ai iā ʻoe kahi hemahema i ʻelua mau ʻano hunaola koko (cytopenia), a i kekahi manawa ʻekolu. Ua kapa ʻia ka hemahema i nā ʻekolu he pancytopenia . ʻO nā kūlana nui ʻekolu e haʻahaʻa ai kēia mau hunaola koko:

• ʻO ka anemia hemolytic autoimmune (AIHA)ʻO kēia ka manawa e hoʻemi ʻia ai nā hunaola koko ʻulaʻula. ʻO ia hoʻi, ua luku ʻia lākou ma mua o ka hiki i ke kino ke hana iā lākou.
• ʻO ka thrombocytopenia pale kino (ITP) : He kūlana kēia kahi e emi ai ka nui o nā platelets. Hoʻouka ka ʻōnaehana pale kino a wehe i nā platelets mai ke koko.
• ʻO ka neutropenia Autoimmune (AIN) : He kūlana kēia e emi ai ka helu o nā neutrophils, kahi ʻano o ke koko keʻokeʻo. Hoʻouka ka ʻōnaehana pale a luku i nā neutrophils.

E noʻonoʻo e pili ana i ia mea, he nui nā kumu no ka haʻahaʻa o ka helu o nā cell koko (cytopenia). I kekahi manawa hiki ke no ka mea ʻaʻole lawa ka hana ʻana o ka iwi iwi i nā cell koko. Eia nō naʻe, i ka cytopenia autoimmune, hoʻopau ka ʻōnaehana pale i kāna mau cell koko ponoʻī.

He aha nā mea hoʻonāukiuki no ka Evans Syndrome?

ʻAʻole maopopo loa i nā kānaka ʻepekema ke kumu o ka ulu ʻana o kēia mau maʻi autoimmune. Akā ua ʻike lākou e hoʻomaka pinepine kēia mau maʻi ma hope o kahi maʻi koʻikoʻi a i ʻole kahi hanana e kau ai i ka nui o ke kaumaha ma ka ʻōnaehana pale. Ua kapa ʻia kēia mau mea he mau mea hoʻoulu .

Eia kekahi, inā loaʻa iā ʻoe kekahi maʻi autoimmune, ʻoi aku ka nui o ka loaʻa ʻana o kekahi maʻi ʻē aʻe ma muli o ka helu helu. Me he mea lā e laha ana kēia maʻi ʻōnaehana pale mai kekahi ʻāpana o ke kino a i kekahi ʻāpana.

Hiki ke loaʻa ka maʻi Evans ma kāna iho (ua kapa ʻia kēia ʻo ka maʻi Evans mua ), a i ʻole hiki ke loaʻa ma ke ʻano he maʻi lua e pili ana i kekahi maʻi ʻē aʻe (ua kapa ʻia kēia ʻo ka maʻi Evans lua) . ʻO kekahi o nā kūlana e hiki ke pili ma kēia ʻano:

• ʻO ka lupus erythematosus ʻōnaehana (lupus)
• ʻO ka hemahema immunololi loli maʻamau
• Ka hemahema IgA koho
• ʻO ka maʻi Sjögren
• ʻO ka lymphoma ʻaʻole Hodgkin
• Leukemia lymphocytic mau loa
• ʻO ka maʻi hepatitis C mau loa
• Ka maʻi HIV mau loa

Inā loaʻa iā ʻoe ka maʻi Evans mua, ua ulu paha ia ma hope o kahi maʻi manawa pōkole, a i ʻole ʻaʻohe ou kumu maopopo. Hoʻokaʻawale nā ​​​​​​kānaka ʻepekema i ka maʻi Evans ma ke ʻano he kūlana idiopathic . ʻO ke ʻano kēia, ʻaʻohe kumu pololei i loaʻa.

He aha nā pilikia e hiki ke loaʻa mai ka Evans Syndrome?

ʻAʻole e loaʻa i nā kānaka a pau me ka Evans Syndrome nā pilikia. Loaʻa i kekahi poʻe nā hōʻailona liʻiliʻi loa. Eia nō naʻe, i nā hihia koʻikoʻi, hiki i kēia mau hemahema o nā cell koko (cytopenias) ke hoʻoweliweli i ke ola. Eia kekahi laʻana:

• Ke lawa ʻole nā ​​hunaola koko ʻulaʻula e lawe i ka oxygen i ke kino, ʻaʻole lawa ka oxygen i loko o ke koko (hypoxemia) . He mea paʻakikī loa kēia i ka puʻuwai a hiki ke alakaʻi i ka maʻi puʻuwai a me ka hāʻule ʻana o ka puʻuwai.
• Ke ʻole lawa kāu mau platelets , ka mea e kōkua ai i ke kāpili ʻana o kou koko, ʻaʻole e kāpili pono kou koko. Hiki i kēia ke hoʻokahe koke iā ʻoe i ke koko. Hiki i kēia ke alakaʻi koke i ka anemia koʻikoʻi.
• Ke nalowale kou kino i nā neutrophils , ka mea e kōkua ai i ke kaua ʻana i ka maʻi, ʻoi aku ka nui o kou loaʻa ʻana i nā maʻi, a hiki iā lākou ke lilo i mea koʻikoʻi a laha ma kou kino. Hiki i kēia ke alakaʻi i kahi maʻi weliweli i kapa ʻia ʻo sepsis .

Pehea e ʻike ai nā kauka i ka Evans Syndrome?

Hoʻomaopopo ʻia ka Evans Syndrome ma o nā hoʻāʻo koko . Hiki ke ʻike ʻia inā loaʻa iā ʻoe nā hōʻailona o ka cytopenia, a i ʻole i ka wā o ka helu ʻana o nā cell koko i hana ʻia i ka wā o ka nānā olakino maʻamau.

Inā hōʻike kāu Helu Koko Piha (CBC) he haʻahaʻa kou mau hunaola koko ʻulaʻula, nā platelet, a i ʻole nā ​​neutrophils, e hana hou aku kāu hui lapaʻau i nā hoʻokolohua. No ka laʻana, e nānā lākou i kou koko no nā pae kiʻekiʻe o nā antibodies e pili ana me ka Evans Syndrome. E nānā pū lākou no nā kūlana ʻē aʻe e hiki ai ke hoʻoulu i ka cytopenia, e like me kekahi mau maʻi a i ʻole ke kanesa.

Ua hōʻoia nā kauka i ka Evans Syndrome ma ka kāpae ʻana i nā kumu ʻē aʻe a pau. I kekahi manawa, pono paha lākou e hana i kahi CT scan (computed tomography scan) a i ʻole kahi biopsy iwi e kāpae i nā kūlana ʻē aʻe.

Aia kekahi lāʻau lapaʻau piha no ka Evans Syndrome? Pehea e mālama ʻia ai?

ʻO ka ʻoiaʻiʻo, ʻaʻohe lāʻau lapaʻau piha no ka Evans Syndrome, a i ʻole kekahi maʻi autoimmune ʻē aʻe. Hoʻōla nā kauka i kēia mau maʻi autoimmune ma ka hāʻawi ʻana iā ʻoe i nā lāʻau lapaʻau e hōʻemi ana i ka hana o kāu ʻōnaehana pale.

Eia kekahi, hiki ke mālama ʻia kāu mau hōʻailona ma ke kaʻawale. No ka laʻana, inā haʻahaʻa loa ka helu o kāu mau cell koko, hiki paha i kāu kauka ke hoʻoponopono i kēia me nā hoʻoili koko i kekahi manawa.

E like me nā maʻi autoimmune lōʻihi, hiki i ka Evans Syndrome ke hoʻomaikaʻi a ʻoi aku ka ʻino i ka hala ʻana o ka manawa. I kekahi manawa me he mea lā e nalowale nā ​​​​hōʻailona (i kapa ʻia he remission) , akā a laila hiki iā lākou ke hoʻi hou a ʻoi aku ka ʻino (i kapa ʻia he relapse ).

Pono paha ʻoe e hoʻāʻo i nā ʻano lāʻau like ʻole i kou ola a pau, no ka mea, hiki ke loli ko lākou holomua i ka hala ʻana o ka manawa. E hoʻāʻo kāu kauka e pale iā ʻoe mai nā pilikia koʻikoʻi loa o ka Evans Syndrome.

He aha nā lāʻau lapaʻau?

ʻO ke ʻano maʻamau no ka Evans Syndrome, ʻo ia nā lāʻau lapaʻau immunosuppressant a, inā pono, nā hoʻoili koko. Hana kēia mau immunosuppressants ma ka hōʻemi ʻana i ka hoʻouka hewa ʻana e kāu ʻōnaehana pale.

ʻO nā ʻano nui o nā lāʻau lapaʻau i hāʻawi ʻia:

• ʻO nā Corticosteroids , e like me ka prednisone . ʻO kēia ka lāʻau lapaʻau mua i hāʻawi ʻia.
• ʻO ka lāʻau lapaʻau immunoglobulin intravenous (IVIg) e pili ana i ka hāʻawi ʻana i kahi hopena e loaʻa ana nā antibodies kanaka ma o kahi aa. Hiki i kēia mau antibodies hou ke hoʻopaʻa i nā antibodies autoimmune hewa a hoʻopau iā lākou.

Inā ʻaʻole ʻoe e pane maikaʻi i nā lāʻau lapaʻau maʻamau, e hoʻāʻo paha kāu kauka i nā lāʻau lapaʻau ʻē aʻe. ʻO kēia mau mea:

• ʻO nā antibodies monoclonal , e like me rituximab . He mau antibodies kēia i hana ʻia i loko o kahi lab. I kekahi manawa hiki i kēia ke hoʻōki i ka pane autoimmune. Hiki i kāu kauka ke kuhikuhi i kēia mau mea inā ʻaʻole holo pono nā lāʻau lapaʻau ʻē aʻe.
• Splenectomy . I nā hihia koʻikoʻi, hiki i kāu kauka ke ʻōlelo aku i ke ʻoki ʻana e wehe i kou spleen. ʻO kou spleen kahi e wehe ʻia ai nā hunaola koko kahiko. No laila, ke wehe ʻia ka spleen, lohi iki ke kaʻina hana.

Aia kekahi mau pilikia a i ʻole nā ​​hopena ʻaoʻao o ka lāʻau lapaʻau?

Ke lawe ʻoe i nā immunosuppressants, ua emi ka pane a kāu ʻōnaehana pale i kāu maʻi autoimmune. Eia nō naʻe, ua emi pū kou pale ʻana i nā maʻi . No laila pono ʻoe e hana i nā hana palekana kūikawā e pale aku ai i ka maʻi.

Hiki i kāu kauka ke ʻōlelo aku i nā lāʻau pale hou aʻe ma waho aʻe o nā mea āu i loaʻa mua ai. Ke nāwaliwali kou ʻōnaehana pale (immunocompromised), ʻoi aku paha kou maʻalahi i nā maʻi āu e loaʻa ʻole ai.

Ke hoʻopilikia nei anei ka noho ʻana me ka Evans Syndrome i ke ola?

ʻAʻole pono ka Evans Syndrome e hoʻopilikia i kou ola. Loaʻa i kekahi poʻe nā hōʻailona liʻiliʻi loa, i kekahi manawa. Eia nō naʻe, hiki i kekahi mau pilikia, e like me ke kahe ʻana o ke koko, ka maʻi puʻuwai, a me ka maʻi, ke hoʻoweliweli i ke ola.

Inā loaʻa iā ʻoe ka maʻi Evans lua, ʻo ia hoʻi, loaʻa iā ʻoe me kahi maʻi maʻi ʻē aʻe, hiki i kēlā ke hoʻopilikia i kou ola. Loaʻa paha iā ʻoe nā pilikia e hoʻoweliweli i ke ola e hele mai me kāu maʻi ʻē aʻe.

He aha ka manaʻolana no kekahi me ka Evans Syndrome?

ʻOkoʻa loa nā ʻike o ka poʻe me ka Evans Syndrome. Hiki ke ʻoluʻolu a koʻikoʻi paha nā hōʻailona. Manaʻo kekahi poʻe ua nalowale loa kā lākou mau hōʻailona (remission). ʻIke hou kekahi iā lākou ma hope (relapse).

He nui ka poʻe e pono ai kekahi ʻano lapaʻau a me nā nānā lapaʻau maʻamau. Hana maikaʻi pinepine nā lāʻau lapaʻau, akā ʻaʻole i nā manawa a pau. I kekahi manawa, ʻike ʻia paha e emi ana ka pono o nā lāʻau lapaʻau i ka hala ʻana o ka manawa. Pono paha ʻoe e hoʻāʻo i nā lāʻau lapaʻau ʻē aʻe.

Pehea e mālama ai iaʻu iho i koʻu wā e noho ana me ka Evans Syndrome?

Inā loaʻa iā ʻoe ʻo Evans Syndrome, he mea nui e hoʻomau i kāu mau hoʻokolohua lapaʻau maʻamau . ʻOiai inā maikaʻi kou manaʻo, makemake kāu kauka e nānā i kou kūlana a ʻike koke inā e ʻoi aku ka ʻino.

Eia kekahi, pono ʻoe e makaʻala mau iā ʻoe iho a makaʻala i nā hōʻailona hou. Inā loaʻa iā ʻoe ke anu ʻaʻole e hoʻomaikaʻi, a inā he kahe koko hiki ʻole ke kāohi ʻia, e kāhea koke i kāu kauka .

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Hiki paha iā ʻoe ke nīnau i kāu kauka i nā nīnau e like me kēia:

• He aha nā ʻano hunaola koko i nele iaʻu?
• ʻEhia ka nui o kā lākou i emi ai?
• He aha nā hōʻailona a i ʻole nā ​​​​hōʻailona hōʻailona e pono iaʻu e makaʻala?
• Pehea e ʻike ai au inā e hana ana ka lāʻau lapaʻau a ʻaʻole paha?
• He aha ka mea e hana inā ʻaʻole holo pono ka lāʻau lapaʻau?
• Āhea wau e hoʻopaʻa ai i kaʻu manawa kauka aʻe?

He maʻi laha ʻole ka Evans Syndrome āu i lohe ʻole ai ma mua. Hiki ke ʻano ʻē a hoʻopilikia nā hōʻailona. ʻO ka hoʻomaopopo ʻana i ka mea e hana nei a me kona manaʻo he hana paʻakikī paha ia.

ʻOiai he maʻi ola ka Evans Syndrome, ʻaʻole like mau ia. E hoʻomau i ka manaʻolana e ʻoi aku kou maikaʻi, ma ka liʻiliʻi i kekahi manawa. ʻOiai ke ʻano o kou manaʻo, eia kāu hui lapaʻau e hāʻawi iā ʻoe i ka mālama āu e pono ai.

Leka Hoʻihoʻi Hope Loa

ʻAe, no laila e hoʻomanaʻo aku kākou iā ʻoe i kekahi o nā mea nui a kākou i kamaʻilio ai.

• He aha ka Evans Syndrome?He maʻi laha ʻole kahi e hoʻouka ai kou ʻōnaehana pale ponoʻī i kou mau hunaola koko ponoʻī.
• Hiki i kēia ke alakaʻi i ka emi ʻana o nā hunaola koko ʻulaʻula ( anemia ), nā platelets (thrombocytopenia), a me nā neutrophils paha (neutropenia) .
• Hiki ke ʻike ʻia nā hōʻailona e like me ka luhi nui, ka keʻokeʻo, ka maʻalahi o ka palapū, ke kahe pinepine ʻana o ke koko, a me nā maʻi pinepine .
• ʻOiai ʻaʻohe lāʻau lapaʻau piha no kēia, hiki ke hoʻomalu ʻia nā hōʻailona me ka lāʻau lapaʻau .
• He mea nui loa ia e hahai i nā ʻōlelo aʻoaʻo lapaʻau maʻamau a e makaʻala i nā hōʻailona.
• ʻAʻole ʻoe hoʻokahi. E kōkua mai kāu mau kauka a me nā poʻe aloha iā ʻoe. He mea nui nō hoʻi e noho maikaʻi.

Manaʻolana wau he mea kōkua kēia ʻike iā ʻoe. Inā he mau nīnau kāu, mai kānalua e kamaʻilio me kāu kauka.

ʻO ka maʻi Evans , ka maʻi autoimmune, cytopenia, anemia, thrombocytopenia, neutropenia, nā maʻi koko