Waldenström macroglobulinemia

ʻO Waldenström macroglobulinemia (WM) kahi maʻi ʻaʻai o nā limfhocytes B (kahi ʻano o ke keʻokeʻo keʻokeʻo). Pili ʻo WM me ka overproduction o nā protein i kapa ʻia ʻo IgM antibodies.

ʻO WM kahi hopena o kahi maʻi i kapa ʻia lymphoplasmacytic lymphoma. He maʻi ʻaʻai kēia o nā hunaola keʻokeʻo, kahi e hoʻomaka ai ka hoʻokaʻawale wikiwiki ʻana o nā hunaola B. ʻO ke kumu kikoʻī o ka hana nui ʻana o ka Anthem IgM ʻike ʻole ʻia. Hiki paha i ka Hepatitis C ke hoʻonui i ka makaʻu o WM. ʻIke pinepine ʻia ka hoʻololi ʻana o ka Gene i nā hunaola B ʻino.

Iecaianoaaiiuo o ka oi IgM nā pale kemikala hiki ke kumu o kekahi mauʻano o nā pilikia:

• ʻO Hyperviscosity, ka mea e mānoanoa ai ke koko. Hiki i kēia ke paʻakikī i ke kahe o ke koko ma o nā kīʻaha koko liʻiliʻi.
• ʻO Neuropathy, a i ʻole nā ​​hōʻino nerve, ke hana a ka pākuʻi IgM me nā aʻa nerve.
• ʻO Anemia, ke nakinaki ka antibody IgM i nā hunaola ʻulaʻula.
• ʻO ka maʻi kīnā, ke waiho ka IgM antibody i loko o ka puʻupaʻa puʻupaʻa.
• ʻO Cryoglobulinemia a me vasculitis (hoʻāhu o nā kīʻaha koko) ke hana ka antibody IgM i nā ʻaoʻao pale pale me ke anuanu.

Kākaʻikahi loa ʻo WM. ʻO ka hapa nui o ka poʻe me kēia ʻano ma luna o 65 mau makahiki.

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

• Kahe ʻana o ka gum a me nā ihu
• ʻImi a hoʻoliʻiliʻi paha ka hihiʻo
• ʻO kaʻili polū i nā manamana lima ma hope o ke anuanu
• Ka piʻo a huikau paha
• ʻUlupā maʻalahi o ka ʻili
• Ka luhi
• Pohā
• Numbness, tingling, a ʻeha ʻeha paha i nā lima, nā wāwae, nā manamana, nā manamana wāwae, nā pepeiao, a me ka ihu
• Rash
• Nūhū pehu
• Ka hoʻohaʻahaʻa kaumaha ʻole
• Lilo ka hihiʻo ma ka maka hoʻokahi

Hiki i kahi hoʻokolohua kino ke hōʻike i kahi ākea, akepaʻa, a me nā pūnana lymph. Hōʻike paha kahi hoʻokolohua maka i ka hoʻonui ʻia ʻana o nā veins i ka retina a i ʻole ke kahe retina (hemorrhages).

Hōʻike kahi CBC i kahi helu haʻahaʻa o nā hunaola ʻulaʻula a me nā platelet. Hōʻike ke kemika o ke koko i ka hōʻike o ka maʻi maʻi puʻupaʻa.

Hōʻike kahi hōʻike i kapa ʻia ʻo ka serum protein electrophoresis i kahi kiʻekiʻe o ka antibody IgM. ʻOi aku ka kiʻekiʻe o nā pae ma mua o 300 milligrams per deciliter (mg / dL), a i ʻole 3000 mg / L. E hana ʻia kahi hōʻike immunzineation e hōʻike i ka loaʻa ʻana o ka antibody IgM mai kahi ʻano pū hoʻokahi, (clonal).

Hiki i kahi hōʻike viscosity serum ke haʻi inā ua mānoanoa ke koko. Kū nā ʻōuli ma ke ʻano he ʻehā manawa mānoanoa o ke koko ma mua o ka maʻamau.

E hōʻike ana kahi biopsy momona o ka iwi i nā helu hoʻonui o nā hunaola ʻē aʻe e like me nā lymphocytes a me nā hunaola plasma.

ʻO nā hoʻokolohua hou aʻe e hana ʻia ana paha:

• 24-hola mimi protein
• Huina protein
• Immun mamanation i ka mimi
• Helu huahelu lymphocyte T (thymus i loaʻa)
• Iwi-kukuna

ʻO kekahi poʻe me WM nāna i hoʻonui i nā antibodies IgM ʻaʻohe o lākou mau ʻōuli. ʻIke ʻia kēia ʻano ʻo smfireing WM. ʻAʻole pono kahi lapaʻau ma mua o ka mālama pono ʻana.

I ka poʻe me nā ʻōuli, mālama ka lāʻau i ka hōʻemi ʻana i nā ʻōuli a me ka makaʻu o ka hoʻomohala ʻana i nā meaola. ʻAʻohe mālama maʻamau maʻamau. Kuhi paha kāu mea mālama ola e komo i kahi hoʻokolohua hoʻokolohua.

Lawe ka Plasmapheresis i nā mea ʻino IgM mai ke koko. Hoʻopili wikiwiki ia i nā ʻōuli i hoʻokumu ʻia e ka mānoanoa o ke koko.

Hiki i nā lāʻau ke komo pū me nā corticosteroids, kahi hui pū ʻana o nā lāʻau chemotherapy a me ka monoclonal antibody i nā pūnaewele B, rituximab.

Hiki ke ʻōlelo ʻia ke kanu ʻōnaehana autologous no kekahi poʻe me ke olakino maikaʻi ʻole.

ʻO ka poʻe i loaʻa ka helu haʻahaʻa o nā ʻulaʻula a keʻokeʻo paha o ke ākea a i ʻole nā ​​platelet e pono ai i ka transfusions a i ʻole nā ​​antibiotic.

ʻO ke ola maʻamau ma kahi o 5 mau makahiki. Noho kekahi poʻe ma mua o 10 mau makahiki.

I kekahi poʻe, hana paha ka maʻi i mau ʻōuli a holo mālie.

Komo pū nā pilikia o WM:

• Hoʻololi i ka hana noʻonoʻo, alakaʻi paha i ka coma
• Hewa puʻuwai
• ʻO ke kahe Gastrointestinal a i ʻole malabsorption
• Nā pilikia ʻike
• ʻO Hives

Kāhea i kāu mea hāʻawi inā ulu nā ʻōuli o WM.

Waldenström macroglobulinemia; Macroglobulinemia - kumu; Lymphoplasmacytic lymphoma; Monoclonal macroglobulinemia

• Waldenström
• Nā Antibodies

Kapoor P, Ansell SM, Fonseca R, et al. ʻIke a me ka hoʻokele o Waldenström macroglobulinemia: Mayo stratification o macroglobulinemia a me ka hoʻoweliweli ʻia (mSMART) alakaʻi 2016. JAMA Oncol. 2017; 3 (9): 1257-1265. PMID: 28056114 pubmed.ncbi.nlm.nih.gov/28056114/.

ʻO Rajkumar SV. Nā maʻi maʻi Plasma. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 178.

Treon SP, Castillo JJ, Hunter ZR, Merlini G. Waldenström macroglobulinemia / lymphoplasmacytic lymphoma. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 87.