He ʻano ʻē anei ka leukemia i hoʻokumu ʻia e ka lāʻau lapaʻau? E kamaʻilio kākou e pili ana i ka Differentiation Syndrome!

Ke mālama ʻia nei ʻoe a i ʻole kekahi āu i ʻike ai no kahi maʻi e like me ka leukemia, hiki iā ʻoe ke loaʻa nā hopena ʻaoʻao i manaʻo ʻole ʻia. I kēia lā, e kamaʻilio mākou e pili ana i ka hui pū ʻana o nā hopena koʻikoʻi e hiki ke hana me nā lāʻau lapaʻau, ʻoiai nā mea i hāʻawi ʻia no ʻelua ʻano maʻi koko. Ua kapa nā kauka i kēia ʻo Differentiation Syndrome . No ka mea hiki ke koʻikoʻi loa, hoʻomaka pinepine nā kauka e mālama i nā hōʻailona ma mua o ka hiki iā lākou ke hōʻoia maoli, hiki ke hōʻemi i ka pilikia o nā pilikia e hoʻoweliweli i ke ola.

He aha ka Differentiation Syndrome?

I ka ʻōlelo maʻalahi, ʻo ka Differentiation Syndrome kahi hui o nā hopena koʻikoʻi i loaʻa i ke kino i kekahi mau lāʻau lapaʻau i hoʻohana ʻia e mālama i ʻelua ʻano maʻi koko, ʻo ia hoʻi ʻo Acute Promyelocytic Leukemia (APL) a me Acute Myeloid Leukemia (AML) . Me he mea lā kekahi o nā cell i loko o ko mākou kino "hele pupule" me kēia mau lāʻau lapaʻau a hoʻomaka e hoʻokuʻu i nā mea he nui i ka manawa hoʻokahi. ʻO kēia ke kumu o kēlā me kēia o nā hōʻailona.

E noʻonoʻo ua ʻike ʻia kekahi hoaaloha o ʻoe me ka APL a hoʻomaka i ka lāʻau lapaʻau. I loko o kekahi mau lā, loaʻa iā ia ke kuni, paʻakikī ka hanu ʻana, a ua pehu iki. I kēlā manawa, manaʻo nā kauka he Differentiation Syndrome kēia.

He aha kēia mau mea hoʻokaʻawale?

ʻO nā mea hoʻokaʻawale he ʻano lāʻau lapaʻau a nā kauka e hoʻohana ai e mālama i ka APL. ʻOi aku ka maʻalahi o ka APL ma mua o nā ʻano AML ʻē aʻe i nā lāʻau lapaʻau e hoʻoulu ai i nā cell e oʻo, a i ʻole e hoʻokaʻawale . Kōkua kēia mau lāʻau lapaʻau i nā cell kanesa (blast cell stage) e ulu i loko o nā cell koko keʻokeʻo maʻamau a olakino. ʻO ia ke kumu e hoʻohana ai nā kauka i kēia mau lāʻau hoʻokaʻawale ma kahi o ka hāʻawi pololei ʻana iā lākou i nā lāʻau chemotherapy .

E like me nā lāʻau lapaʻau maʻi kanesa ʻē aʻe, kōkua kēia mau lāʻau i ka hoʻōki a hoʻolohi paha i ka ulu ʻana o nā hunaola kanesa. Hiki i nā kauka ke hoʻohana wale i kēia mau mea hoʻokaʻawale, me ka hui pū ʻana me kekahi, a i ʻole me ka chemotherapy. Hiki iā lākou ke mālama pono i ka APL. Eia nō naʻe, hiki i kekahi poʻe me APL ke loaʻa nā hopena ʻaoʻao koʻikoʻi mai kēia mau lāʻau.

He aha nā lāʻau lapaʻau e hiki ke hana i ka Differentiation Syndrome?

Hiki ke hana ʻia kēia kūlana e nā mea hoʻokaʻawale i hāʻawi ʻia i ka APL. ʻO nā hiʻohiʻona penei:

• ʻO ka waikawa All-trans-retinoic (ATRA) kahi ʻano o ka huaora A.
• ʻO ka Arsenic Trioxide (ATO) kahi ʻano o ka arsenic.

Eia kekahi, no kekahi poʻe me AML, nā kaukaʻO nā mea hoʻopaʻa Isocitrate Dehydrogenase (IDH) kahi papa o nā lāʻau chemotherapy i hoʻohana ʻia. Hiki i kēia mau mea hoʻopaʻa IDH (ivosidenib) a me (enasidenib) ke hana i ka Differentiation Syndrome. ʻAʻole wale kēlā, akā hiki i nā mea hoʻopaʻa FLT3 (e.g. (midostaurin) , (gilteritinib) ) ke hana i kēia maʻi.

ʻO wai ka mea e pono ai nā mea hoʻopaʻa IDH?

• Nā kānaka i hoʻi hou mai ko lākou maʻi kanesa.
• ʻO nā kānaka me ke kanesa paʻakikī e mālama.
• ʻO nā kānaka me kahi hoʻololi gene kikoʻī (IDH1 a i ʻole IDH2 mutation).
• ʻO ka poʻe hiki ʻole ke ʻae i ka lāʻau chemotherapy maʻamau.

ʻO wai ka mea i ʻoi aku ka nui o ka loaʻa ʻana o ka Differentiation Syndrome?

He haʻahaʻa ka helu o nā keʻena koko keʻokeʻo (WBC) o ka nui o ka poʻe me APL. Eia nō naʻe, ʻoi aku ka nui o ka pilikia o ka poʻe me ka helu WBC kiʻekiʻe i ka hoʻomohala ʻana i ka Differentiation Syndrome. I kēia mau hihia, kuhikuhi pinepine nā kauka i kahi lāʻau i kapa ʻia ʻo Prednisone a i ʻole Dexamethasone e hōʻemi i ka pilikia o kēia syndrome.

Pehea ka maʻamau o ka Differentiation Syndrome?

He ʻano maʻi laha ʻole kēia. ʻO AML ke ʻano maʻamau o ka leukemia i nā pākeke, ʻoiai he 1% ia o nā maʻi kanesa āpau. Ma ʻAmelika Hui Pū ʻIa, loaʻa ka APL ma kahi o hoʻokahi i loko o 250,000 poʻe.

Mai ka poʻe me AML a me APL:

• Ma kahi o 25% o nā maʻi APL i loaʻa nā lāʻau ATRA a me ATO ua hoʻomohala i ka Differentiation Syndrome.
• Ua hōʻike ʻia kēia ʻano ma waena o 14% a me 19% o nā maʻi AML e lawe ana i nā mea hoʻopaʻa IDH.

He aha ke kumu o kēia Differentiation Syndrome?

ʻAʻole maopopo i nā kānaka noiʻi ke kumu e pane ai kekahi poʻe i ka mālama ʻana i ka APL ma kēia ʻano. Hōʻike nā noiʻi mua e hoʻoulu nā lāʻau lapaʻau kanesa i nā cell leukemia e hoʻokuʻu koke i nā kemika o ka ʻōnaehana pale. Ua kapa ʻia kēia ʻo cytokine release syndrome . Hoʻoulu kēia mumū i kāohi ʻole ʻia i nā ʻano hōʻailona like ʻole, mai ka ʻoluʻolu a i ka koʻikoʻi.

He aha nā hōʻailona o ka Differentiation Syndrome?

ʻO ka maʻamau, hoʻomaka ke kanaka me APL e hoʻomohala i nā hōʻailona o ka Differentiation Syndrome i loko o hoʻokahi a ʻelua paha pule o ka hoʻomaka ʻana o ATRA a/a i ʻole Arsenic Trioxide. Eia nō naʻe, inā ʻoe e lawe ana i ka lāʻau lapaʻau IDH inhibitor no AML, hiki ke lōʻihi iki aʻe ka manawa e ʻike ʻia ai nā hōʻailona.Hiki ke nalo aku (hiki ke ʻike ʻia kēia mau hōʻailona mai hoʻokahi lā a ʻelima a ʻeono mahina ma hope o ka hoʻomaka ʻana e hoʻohana i ka lāʻau).

ʻO kēia nā hōʻailona i ʻike pinepine ʻia:

• Kuke .
• Ka hōʻiliʻili ʻana o ka wai keu a puni kou puʻuwai a me kou māmā (Pleural Effusion) .
• Ka hāʻule ʻana o nā puʻupaʻa .
• Ke koko haʻahaʻa (Hypotension) .
• Haʻahaʻa ka pae oxygen i loko o ke koko (Hypoxemia) .
• Ka paʻakikī o ka hanu ʻana (Dyspnea) .
• Ka pehu ʻana o nā lima, nā wāwae, a me ka ʻāʻī.
• Kuni me ke kumu ʻole.
• Piʻi kaumaha me ke kumu ʻole.

He mea nui: Inā loaʻa iā ʻoe kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​ʻaʻole ia he manaʻo he loaʻa iā ʻoe ka Differentiation Syndrome. Eia nō naʻe, he mea nui e hoʻomaopopo koke i kāu kauka inā ʻike ʻoe i kekahi o kēia mau hōʻailona.

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

Hiki i kēia kūlana ke alakaʻi i kekahi manawa i nā kūlana koʻikoʻi loa. Eia kekahi laʻana:

• Ka hāʻule ʻana o ka puʻuwai .
• Ka hāʻule ʻana o nā puʻupaʻa .
• Ka hāʻule ʻana o ka māmā .
• Ke kahe ʻana o ke koko mai loko mai o nā māmā (Pulmonary Hemorrhage) .
• Pneumonia .
• Sepsis ( kahi maʻi koʻikoʻi e laha ana ma ke kino holoʻokoʻa).

Inā ʻike ʻoe i kekahi o kēia mau hōʻailona, ​​e kāhea koke i kāu kauka . Inā pilikia ʻoe i ka hanu ʻana a i ʻole ka ʻeha o ka umauma, e kāhea koke i ka 911 (a i ʻole kāu helu ulia pōpilikia kūloko) . ʻO ka poʻe me APL e mālama pinepine ʻia ma ka haukapila a hiki i ka hala ʻana o ka pilikia o ka differentiation syndrome.

Pehea e ʻike ʻia ai ka Differentiation Syndrome?

Hiki ke paʻakikī ke ʻike i kēia kūlana i kekahi me APL, no ka mea, he nui nā kūlana ʻē aʻe e hiki ke hana i nā hōʻailona like. I nā hihia koʻikoʻi, hoʻomaka paha nā kauka e mālama i nā hōʻailona i ka wā e manaʻo ai lākou he Differentiation Syndrome, ma mua o ka hiki iā lākou ke hōʻoia ʻo ia ka mea kūpono.

E paipai kāu kauka i kekahi mau hoʻokolohua e hōʻoia i ke kūlana a kāpae i nā kūlana olakino ʻē aʻe. Hiki i kēia mau mea ke komo pū me:

• Helu Koko Piha (CBC) .
• X-ray o ka umauma .
• Echocardiogram (kahi hoʻāʻo e nānā ai i ka hana o ka puʻuwai).
• ʻO ke kiʻi CT umauma .
• ʻO ka Bronchoscopy (kahi hoʻāʻo e hoʻohana ana i kahi kāmela e nānā i loko o nā māmā).
• Nā hoʻāʻo koko ʻē aʻe (Bloodwork).

Aia kekahi lāʻau lapaʻau no ka Differentiation Syndrome?

I ka hapanui o nā hihia, hiki i ka poʻe me ka leukemia promyelocytic acute (APL) ke ho'ōla piha mai ka maʻi differentiation inā loaʻa iā lākou ka lāʻau lapaʻau koke a kūpono . ʻO ka APL i kēia manawa he maʻi hiki ke ho'ōla nui ʻia. No laila, ʻaʻohe mea e makaʻu ai.

Pehea e mālama ʻia ai ka Differentiation Syndrome?

Hoʻomaʻamaʻa pinepine nā kauka i nā hōʻailona o ka differentiation syndrome me kahi lāʻau corticosteroid a glucocorticoid paha , e like me dexamethasone . I nā hihia koʻikoʻi, hiki i kāu kauka ke ʻōlelo aku e hoʻōki i kāu mālama ʻana i ka maʻi kanesa a hiki i ka pau ʻana o kāu mau hōʻailona.

Hoʻōla ʻia ka hapa nui o ka poʻe me APL ma ka haukapila a hiki i ka hala ʻana o ka pilikia o ka differentiation syndrome. Inā he outpatient ʻoe a ke kānalua ʻia nei ka differentiation syndrome, hiki i kāu mau kauka ke hoʻoholo e mālama iā ʻoe ma ka haukapila i ka pō no ka hoʻokele a nānā ʻana paha. E kōkua kāu hui lapaʻau e hōʻoia i kāu hōʻailona a mālama pono i nā kūlana e hoʻoulu ai i kāu mau hōʻailona.

He aha kaʻu e hana ai e hōʻemi i koʻu pilikia o ka hoʻomohala ʻana i ka Differentiation Syndrome?

ʻAʻohe mea hiki iā ʻoe ke hana e hōʻemi i kou pilikia o kēia maʻi. Inā ʻoe e lawe ana i ka chemotherapy a i ʻole nā ​​​​lāʻau lapaʻau kanesa ʻē aʻe, e haʻi koke i kāu kauka e pili ana i nā hōʻailona āu e ʻike ai . Eia kekahi, e makaʻala i nā hōʻailona e makaʻala ai (e like me kā mākou i kūkākūkā ai ma luna) a kamaʻilio me kāu kauka inā he mau hopohopo kāu. ʻO kēia ka mea nui loa.

He aha ka manaʻolana no ka Differentiation Syndrome?

ʻO ka manaʻo no ka maʻi differentiation ma ke ʻano laulā he maikaʻi ia no ka hapa nui o nā kānaka. I ka hapanui o nā hihia, hiki iā ʻoe ke hoʻomau i kāu lāʻau anticancer, i ka wā a ma hope paha o ka lāʻau lapaʻau me nā steroids. E kamaʻilio kāu hui lapaʻau me ʻoe e pili ana i kāu mau koho lapaʻau.

I ka manawa hea ʻoe e ʻimi ai i ke kōkua lapaʻau no ka Differentiation Syndrome?

Inā ʻike ʻoe i kekahi mau hōʻailona o ka maʻi differentiation i ka wā e mālama ʻia ai ka maʻi kanesa, e kāhea koke i kāu kauka . He mea paʻakikī paha iā ʻoe a i ʻole kāu kauka ke ʻike inā ua hoʻokumu ʻia kāu mau hōʻailona e ka mālama ʻana i ka maʻi kanesa a i ʻole kekahi ʻano ʻē aʻe. Hiki i ka mālama mua ke kōkua i ka hōʻoluʻolu ʻana i nā hōʻailona a hoʻemi i ka pilikia o nā pilikia.

Hiki ke paʻakikī ka mālama ʻana i ka maʻi kanesa. Eia nō naʻe, hiki ke hoʻonāwaliwali ke ʻike he mau hopena ʻaoʻao ko ka lāʻau ponoʻī. ʻO ka Differentiation Syndrome kahi hui o nā hopena e hoʻoweliweli ai i ke ola i nā lāʻau lapaʻau maʻi koko. Eia nō naʻe, hiki ke mālama ʻia inā ʻike ʻia a mālama ʻia i ka wā mua . No ka mea hiki ke ʻoi aku ka ʻino o nā hōʻailona, ​​​​hiki i nā kauka ke hoʻomaka e mālama iā ʻoe ma mua o ka hōʻoia ʻana o kāu mau hopena hoʻāʻo he Differentiation Syndrome kou. Hiki i ka mālama lapaʻau mua ke kōkua i ka hōʻoluʻolu i kāu mau hōʻailona a hoʻihoʻi iā ʻoe ma ke ala e hoʻōla ai.

Leka Lawe-Home

• He hopena koʻikoʻi ka Differentiation Syndrome i hiki ke kū mai ma muli o kekahi mau lāʻau lapaʻau i hāʻawi ʻia no nā ʻano leukemia i kapa ʻia ʻo APL a me AML.
• Hiki ke ʻike ʻia nā hōʻailona e like me ke kuni, ka paʻakikī o ka hanu ʻana, a me ka pehu ʻana o ke kino.
• Inā ʻike ʻoe i kēia mau hōʻailona, ​​​​​​he mea nui e hoʻomaopopo koke i kāu kauka .
• Inā ʻike koke ʻia a mālama ʻia me nā lāʻau lapaʻau e like me Dexamethasone, hiki ke ho'ōla pinepine ʻia.
• Mai hopohopo ke lohe ʻoe i kēia. E kōkua kāu hui lapaʻau iā ʻoe. ʻO ka mea nui loa, ʻo ia ka hoʻomaopopo ʻana iā lākou e pili ana i nā loli, nā hōʻailona.

ʻO ka maʻi differentiation, leukemia, ka mālama ʻana i ka maʻi kanesa, nā hopena ʻaoʻao, APL, AML, ATRA, nā hōʻailona