He aha ka Lymphoplasmacytic Lymphoma?

Anter

• LPL vs. nā lymphomas ʻē aʻe
• He aha ka hopena i ke kikowaena pale?
• He aha nā hōʻailona?
• He aha ke kumu?
• Pehea ia ʻike?
• Nā koho lapaʻau
• E nānā a kali
• ʻO Chemotherapy
• Lapaʻau olaola
• Pahuhopu kau ʻia
• Mea hoʻoliʻiliʻi keleawe
• Nā hoʻāʻo hoʻokolohua
• He aha ke kuanaʻike?

Hōʻike Overview

ʻO Lymphoplasmacytic lymphoma (LPL) kahi ʻano laha ʻole o ka maʻi ʻaʻai e ulu lohi a hoʻololi i ka hapanui o nā mākua. ʻO ka makahiki maʻamau i ʻike ʻia he 60.

ʻO nā Lymphomas nā maʻi ʻaʻai o ka ʻōnaehana lymph, kahi ʻāpana o kāu ʻōnaehana pale e kōkua i ke kaua aku i nā maʻi. I ka lymphoma, nā ʻula koko keʻokeʻo, nā limfositita B a i ʻole nā ​​lymphocytes T, ulu aʻe ma waho o ka kaohi no ka hoʻololi. I ka LPL, hana kolohe nā lymphocytes B maʻamau i loko o kou iwi iwi a hoʻohuli i nā hunaola maikaʻi.

Aia ma kahi o 8.3 mau hihia o LPL no 1 miliona mau kānaka ma United States a me Western Europe. ʻOi aku ka maʻamau i nā kāne a me nā Caucasians.

LPL vs. nā lymphomas ʻē aʻe

Hoʻomaopopo ʻia ka lymphoma o Hodgkin a me ka lymphoma non-Hodgkin e ke ʻano o nā hunaola e lilo i maʻi ʻaʻai.

• Loaʻa i nā lymphomas o Hodgkin kahi ʻano kikoʻī o ke keena ʻino i kū ʻia i kēia manawa, i kapa ʻia ʻo Reed-Sternberg cell.
• Hoʻomaopopo ʻia nā ʻano nui o nā lymphomas non-Hodgkin e kahi e hoʻomaka ai nā maʻi ʻaʻai a me nā genetics a me nā ʻano ʻē aʻe o nā hunaola malignant.

ʻO LPL kahi lymphoma non-Hodgkin e hoʻomaka i nā lymphocytes B. He lymphoma laha ʻole loa ia, ma kahi o 1 a 2 pakeneka wale nō o nā lymphomas āpau.

ʻO ka ʻano maʻamau o ka LPL ʻo Waldenström macroglobulinemia (WM), kahi ʻano e kahi hana pono ʻole o ka immunoglobulin (anti-antibodies). Kuhi hewa ʻia ʻo WM i kekahi manawa e like me LPL, akā he ʻāpana maoli ia o LPL. Ma kahi o 19 mai 20 mau kānaka me LPL i loaʻa ka immunoglobulin abnormalities.

He aha ka hopena i ke kikowaena pale?

Ke hana ʻo LPL i nā lymphocytes B (nā hunaola B) e hoʻohua nui i loko o kou iwi iwi, ʻoi aku ka liʻiliʻi o nā kikoʻī koko e hana ʻia.

ʻO ka maʻamau, neʻe nā pūnaewele B mai kou iwi iwi a i kou spleen a me nā lymph node. Ma laila, lilo paha lākou i mau hunaola plasma e hana ana i nā mea pale e hakakā ai i nā maʻi. Inā ʻaʻole lawa kāu mau pūnaewele koko maʻamau, hoʻopilikia ia i kāu ʻōnaehana pale.

E hopena paha kēia i:

• anemia, ka nele o nā hunaola ʻulaʻula
• neutropenia, kahi hemahema o kekahi ʻano o ke keʻokeʻo koko keʻokeʻo (i kapa ʻia he neutrophils), kahi e hoʻonui ai i ka maʻi maʻi
• thrombositopenia, kahi hemahema o nā platelet koko, kahi e hoʻonui ai i ke kahe ʻana o ke kahe a me ka ʻeha ʻana i nā pilikia

He aha nā hōʻailona?

ʻO LPL kahi maʻi maʻi lohi e ulu lohi ana, a ma kahi o ka hapakolu o ka poʻe me ka LPL ʻaʻohe o lākou mau hōʻailona ma ka manawa a lākou e ʻike ai.

Aia i ka 40 pākēneka o ka poʻe me LPL i kahi ʻano anemia akahai.

Pili pū kekahi mau ʻōuli ʻē aʻe o LPL:

• nāwaliwali a nāwaliwali (pinepine ʻia e ka anemia)
• fiva, sweats i ka pō, a me ka pohō kaumaha (pili pinepine ʻia me B-cell lymphomas)
• ʻike hihi ʻole
• ʻūpā
• kahe ka ihu
• gums kahe
• ʻōpala
• hoʻokiʻekiʻe ʻia beta-2-microglobulin, kahi hōʻailona koko no nā tumors

Ma kahi o 15 a 30 pakeneka o ka poʻe me LPL i:

• pehu lymph nodes (lymphadenopathy)
• ka hoʻonui ʻana o ke ake (hepatomegaly)
• spleen hoʻonui (splenomegaly)

He aha ke kumu?

ʻAʻole maopopo maopopo ke kumu o LPL. Ke ʻimi nei nā mea noiʻi i kekahi mau mea hiki.

• Aia paha he ʻāpana kūpuna, e like me 1 ma ka 5 mau kānaka me WM he hoahānau nona ka LPL a i ʻole kekahi ʻano like lymphoma.
• Ua ʻike kekahi mau noiʻi e pili ana paha ka LPL me nā maʻi autoimmune e like me Sjögren Syndrome a i ʻole me ka maʻi hepitis C, akā ʻaʻole hōʻike nā noiʻi ʻē aʻe i kēia loulou.
• Loaʻa ka poʻe me ka LPL i kekahi ʻano hoʻololi kūʻē i hoʻoili ʻole ʻia.

Pehea ia ʻike?

He paʻakikī ka hōʻailona o LPL a hana ʻia hoʻokahi ma hope o ka haʻalele ʻana i nā hiki ʻē aʻe.

Hiki i ka LPL ke hoʻohālikelike me nā lymphomas B-cell ʻē aʻe me nā ʻano like o ka hoʻokaʻawale ʻana o ka cell plasma. Pākuʻi ʻia kēia mau mea:

• mantle cell lymphoma
• leukemia lymphocytic mau / lymphocytic lymphocytic mau
• lymphoma ʻāpana palena
• plasma cell myeloma

E nānā kino kāu kauka iā ʻoe a nīnau i kāu mōʻaukala. E kauoha lākou i ka hana o ke koko a malia paha he iwi iwi a biopsy lymph node e nānā i nā hunaola ma lalo o kahi microscope.

Hiki paha i kāu kauka ke hoʻohana i nā hoʻokolohua ʻē aʻe e kāpae i nā kanesa like a hoʻoholo i ke kahua o kāu maʻi. Pākuʻi ʻia paha kēia i kahi X-ray umauma, CT scan, PET scan, a me ultrasound.

Nā koho lapaʻau

E nānā a kali

ʻO LBL kahi maʻi maʻi lohi e ulu lohi ana. E hoʻoholo paha ʻoe a me kāu kauka e kali a nānā i kou koko ma mua o ka hoʻomaka ʻana o ka lāʻau. Wahi a ka American Cancer Society (ACS), ʻo ka poʻe lohi i ka mālama ʻana a hiki i ka wā e pilikia ai kā lākou mau ʻōuli, loaʻa ka lōʻihi o ke ola e like me ka poʻe e hoʻomaka i ka lāʻau ke loaʻa koke iā lākou.

ʻO Chemotherapy

Hiki ke hoʻohana ʻia i kekahi mau lāʻau i hana i nā ʻano like ʻole, a i ʻole nā ​​hui pū ʻana o nā lāʻau, e pepehi i nā hunaola maʻi ʻaʻai. Pākuʻi ʻia kēia mau mea:

• chlorambucil (Leukeran)
• fludarabine (Fludara)
• bendamustine (Treanda)
• cyclophosphamide (Cytoxan, Procytox)
• dexamethasone (Decadron, Dexasone), rituximab (Rituxan), a me cyclophosphamide
• bortezomib (Velcade) a me rituximab, me ka ʻole o ka dexamethasone
• cyclophosphamide, vincristine (Oncovin), a me prednisone
• cyclophosphamide, vincristine (Oncovin), prednisone, a me rituximab
• thalidomide (Thalomid) a me rituximab

E loli ka regimen kikoʻī o nā lāʻau, kaukaʻi ʻia i kou olakino laulā, kāu mau ʻōuli, a me nā hana e hiki mai ana i kēia wā.

Lapaʻau olaola

ʻO nā lāʻau lapaʻau biological therapy nā mea hana i hana ʻia e like me kāu ʻōnaehana pale ponoʻī e pepehi i nā hunaola lymphoma. Hoʻohui ʻia paha kēia mau lāʻau me nā lāʻau ʻē aʻe.

ʻO kekahi o kēia mau antibodies i hana ʻia e kānaka, i kapa ʻia he monoclonal antibodies, he:

• rituximab (Rituxan)
• ofatumumab (Arzerra)
• alemtuzumab (campath)

ʻO nā lāʻau olaola ʻē aʻe nā lāʻau lapaʻau immunomodulate (IMiDs) a me nā cytokine.

Pahuhopu kau ʻia

Hoʻopaʻa nā lāʻau lapaʻau i māka ʻia e pale i nā loli paʻa e hopena ai i ka maʻi ʻaʻai. Ua hoʻohana ʻia kekahi o kēia mau lāʻau e hakakā i nā maʻi ʻē aʻe a ʻimi ʻia nei no LBL. Ma ka laulā, pale kēia mau lāʻau i nā protein i ʻae i nā hunaola lymphoma e ulu mau.

Mea hoʻoliʻiliʻi keleawe

ʻO kēia kahi lāʻau hou i ʻōlelo ʻia e ka ACS he koho paha no ka poʻe ʻōpio me LBL.

Ma ka laulā, lawe ʻia nā hunaola kūmole koko mai ke kahe o ke koko a mālama paʻahau ʻia. A laila hoʻohana ʻia kahi lāʻau kiʻekiʻe o ka chemotherapy a i ʻole ka radiation e hoʻomake i nā hunaola iwi āpau (maʻamau a maʻi ʻaʻai), a hoʻihoʻi ʻia nā hunaola koko i ke kahe o ke koko. Hiki mai nā hunaola koʻokoʻo mai ke kanaka e mālama ʻia (autologous), a i ʻole hāʻawi ʻia ʻia e kekahi e kūlike kokoke i ke kanaka (allogenic).

E makaʻala i nā transplants cell stem i kahi wā hoʻokolohua. Eia kekahi, aia nā hopena hopena pōkole a me ka wā lōʻihi mai kēia mau transplants.

Nā hoʻāʻo hoʻokolohua

E like me nā ʻano like ʻole o ka maʻi ʻaʻai, ke hoʻomohala ʻia nei nā mea hou, a ʻike paha ʻoe i kahi hoʻokolohua hoʻokolohua e komo ai. E nīnau i kāu kauka e pili ana i kēia a kipa iā ClinicalTrials.gov no ka ʻike hou aku.

He aha ke kuanaʻike?

ʻAʻohe o LPL e ola ai. Hele paha kāu LPL i ke kala kala akā hoʻi ma hope. Eia kekahi, ʻoiai he maʻi kolo e ulu lohi ana, i kekahi mau hihia hiki ke lilo i mea ʻoi aku ka ikaika.

Hoʻomaopopo ka ACS he 78 pākēneka o ka poʻe me LPL e ola nei i ʻelima mau makahiki a ʻoi.

Ke hoʻomaikaʻi nei nā helu ola no ka LPL i ka hoʻomohala ʻia ʻana o nā lāʻau hou a hoʻomohala ʻia nā lāʻau hou.