Waldenstrom ka Macroglobulinemia: symptoms, ke kumu, prognosis

Hereditary koko maʻi i pohihihi, akā, ua noho pilikia loa ana i kānaka. Kekahi mau maʻi o Vandelstrema macroglobulinemia.

Ma mua o ka mea, ua wehewehe aku?

Ma lalo o kēia maʻi i loko o ke kālā no lapaau pili i ka hereditary hematological maʻi pale pu ma ke alo o ke koko macroglobulin. Kēia kumuʻiʻo ua hana ma ka ha awina o monoclonal B-lymphoid koko aeee (B-lymphocytes).

Maʻamau, mau keena i kuleana no ka hoʻololi kemikala o ke koko-M globulin. I ka wā i kekahi hana o ka iwi lolo (pinepine i loko o ka ulu ana o kaʻeho kaʻina) o ka loli ma ka'aminoʻakika haku mele 'ana a me ka kaʻina o ka'ōewe, e alakai i ka hoʻololi kemikala macroglobulin, a laila ulu Waldenstrom ka macroglobulinemia.

Ka maʻi Ua loa pohihihi keia, ma kahi o 3 hihia no miliona kanaka. Ua hoʻomōhala ana iʻano nui i loko o nā kānaka ma luna o ka makahiki o 55 makahiki. keia pathology ua aneane 'aʻole e hana ana i ka 40 makahiki.

Pehea, alaila, hoike mai i ka maʻi ma ka ke kelepona hele, a'aʻaʻa pae? I nā hale i loli like me ka hopena ma laila i loko o ka wahi mua a me ka ia?

Ke pathogenesis o ka maʻi

Ma ka naau o ka patogenza ka maʻi mea he malignant ka hoʻonuiʻana o ka wai koko keena clone keena hiki ke ulu immunoglobulin M. kēia mau keena hele io lākou lā i ka iwi lolo, akepaa, spleen, kahi e hoʻomaka i ka 'Imi proliferate a pee pathogenic macroglobulin. Pela i ulu Waldenstrom ka macroglobulinemia.

Ke hoʻomāhuahua o pathogenic kumuʻiʻo i loko o ke koko hiki aku ai i ka ulu ana o kona (viscosity). E like me ka hopena o ka hoemi koko kahe ana ma ka moku, a hiki aku ai i kona stagnation. Eia hou kekahi, inhibited wai koko coagulation kumumea (aie i ka hoʻi i wahīʻia, a inactivate ia macroglobulins). No ka mea, o keia o Nyos i ka maʻamau clot kahua, a ultimately hiki aku ai i mahuahua kahe ana ke koko.

Visually, ka wā hookolokolo ma lalo o ka microscope, ka iwi lolo keena, hiki ke ike i laila accumulations "plazmatizirovannyh" lymphocytes, he nui helu o oʻo B-lymphocytes, a pee hoʻoʻino uao o ke kia keena. A pau o kēia mau keena paipai ia he mea he Waldenstrom macroglobulinemia.

symptoms

Pehea hiki clinically e mahuʻi mua o kēia maʻi?

Ka mua nā mea maʻi e ohumu o nonspecific symptoms - mau nawaliwali, haʻahaʻa-papa kuni, no ka lōʻihi manawa, sweating, ke kaupaona poho noʻaʻohe'ē aʻe kumu.

I ka papa kuhikuhiE symptom, ma ke kumu o ka a, ua hiki hāʻupu aʻe i Hawaiʻi Vandelstrema macroglobulinemia, ua mahuahua kahe ana ke koko mai ka ihu, nui aʻe ana, a mucous membranes. Loa emi pinepine mālama subcutaneous paopao koke mai oia a me paopao koke mai oia.

Over manawa, e hoʻomōhala hepatosplenomegaly a me ka lymphadenopathy (pehu lymph aka wahi). Ua mea no hoi e hiki i ka ulu ana o complications macroglobulinemia.

I ka hoʻokō 'ole' ana i ka hoʻolako 'āwīwī mālama' aia no he pilikia o ka coma ahonui, a me ka make (akā, e make nā mea maʻi e IeAUPIIe, IAa IO,ʻaʻole no ka nui loa ma luna o nā mea a lākou i loaʻa mai Waldenstrom ka macroglobulinemia). Ke kumu o ko lākou make nō i ka hopena o ka ulu complications.

complications o ka maʻi

I ka ia hiki ke huikau e ka maʻi?

Kuhikuhi 'ole i loko o ka pathological kaʻina hana o ka mea'ōnaehana mānowai koko'ōnaehana, o ka ipu uuku - capillaries, arterioles a me venules. Ma ka hoemi koko malama i ka hooulu ana o ka retinopathy, nephropathy. Hakuʻala ka pōʻino. Ma keia hihia wehewehe aku ma ka hooulu ana o glomerular vascular occlusion a me ka urolithiasis me ka palapala moʻopane o ka urate.

Macroglobulinemia Waldenstrom wehewehe aku ma ka haahaa e hoʻonele a me ka hui opportunistic nā mea palahēhē complicating a me ka lapaʻau 'ana no ka mea nń ku maʻi.

E like me ka Holomua o ke kaʻina hana i na hiohiona suppression oʻaneʻane a pau germs o hematopoiesis a me ka hooulu ana o ka pancytopenia. Amyloidosis wehewehe aku ma ke ana a me ka pathological lesions E like me ka kumuʻiʻo ake a me na kiaha spleen koko, i hou worsens a no laila lākouʻole kuleana pili i.

lapaʻau '

He aha metrics e kōkuaʻoe ike i ka loli Hawaiʻi macroglobulinemia?

Mua, mākou e hoʻolohe i ka CBC. e hoomahuahua i ka papa kuhikuhiE nā hoailona o ka maʻi i loko o ka erythrocyte sedimentation ana, a me ke kahua o ka hana i ho'ākāka ' "rouleaux" - sticking pu o ka ula koko keena. Wbc nō kāu Hōʻike ka hoonuiia ka helu ana o lymphocytes ma ka Ikepili a me ka helehelena o kaʻiliahi leukocytes 'ano apau loa.

E hōʻoia i kaʻike, ma ka mālama nei immuno. Ma hope o ka hoʻokō 'ia o kēia ao hiki ole e hōʻike ana i ka mahuahua nui i loko o ke koko immunoglobulin M

Monoclonal immunoglobulins i hoʻoholo i loko o ke koko ma hope o ka hou o ka 'ae' sera.

Additional, akā,ʻaʻole i ho'ākāka 'symptoms loaʻa i hoonee aku i ake a me ka spleen, ka loaʻaʻana o ka amyloid ma biopsy kappillyarah hakuʻala, e like me hoemi i ka helu o ka wai koko coagulation kumumea (i nui, ololi 8).

lapaʻau

I ka kuʻina kakahiaka nui o ka maʻi, ke ole ka mea, he nui na hui, komo, 'ilepili lapaʻau uaʻaʻole i hōʻike. Pinepine, i kēia mau nā mea maʻi i ma lalo o 'inikua hooponopono ma ka Apana hematologist.

I ka na symptoms mua a me ka hooiaio ana i ka maʻi - Waldenstrom macroglobulinemia, hoʻomaka kekahi Inc me cytostatics. No ka lapaʻau i ai 'e like me "hlorbutin", "Cyclophosphamide". Ka mea, i ka cytostatic ia, a uhi i ka ha awina o ka malignant clone o B-lifotsitov.

Hoonohoia ma ka mua wahi "hlorbutin" i 6 mg kela la i keia hōʻike waha no 3-4 pule. Mahuahua ka mahele lāʻau mea fraught me ka hooulu ana o ka iwi lolo aplasia. Ma hope o hoʻopau i ka papa o ka iapaau ana i hoakaka ia mālama 'ana i nā māhele o ka lāʻau (4.2 mg) kela'ē aʻe lā.

No ka hoʻoikaika 'ana i ke koko rheological kiko'î i hoʻohana plasmapheresis. plasmapheresis lapaʻau ua lawe mai no ka pono o cytotoxic ai '. No ka mea wehe kaʻina hana i hoikeia ma 2 nā lika wai koko me kona mau pou hakahaka ma kona inactivated.

Kāohi

No ka mea, o ka maʻi i hereditary, ia mea paakiki ia ma kekahi ala lakou aloha ia kona ulu ana, no ka mea, ua mua ia e waiho i loko o nā nā'ōewe. Na wale ala, e pale i ka hooulu ana o ka 'eleuʻike, o ka maʻi a me ka pono-e holo ana kumumanao o ka lapaau.

Kahiko o ka'āina pili i ka maʻi hiki e paha i ke ola i ke Aloha Hawaii, e haawi ana i ka pūnuku puhi a me ka waiʻona, kali paka a me spicy mau kiaha.

Mai ka maʻi mea aaiaoe, ka mea hiki ke kāhea kona ulu ana a me kekahi mau hewa ana - hana me ka aniline make, pena, varnishes.

Pinepine mea hoʻomaʻi nā mea palahēhē mea i hiki ke hoʻololi i ka kanaka genome. Kakahiaka lapaʻau o kēia mau maʻi, e kōkua pale loli i nā'ōewe, a pale aku i ka ulu ana o ka macroglobulinemia.

Kāohi ana hiki ke wahi e familiarize nā mea maʻi i nā hiʻona o ka mai, a me ka paipai 'ana i kā lākou mau hana, e hooko i ke ola i ke Aloha Hawaii.

prognosis

He aha ka iini nui i nā mea maʻi i ua ulu Waldenstrom macroglobulinemia? Prognosis o ka maʻi hilinaʻi nui ma luna o ka oolea o ka maʻi i i ulu complications a me ka 'eleu lapaʻau.

Inā i ka maʻi i loko o kona kakahiaka kahua o ka? Acaeoey, ma ka hapanui o na hihia ia mea hiki ke ole kona holomua. Oia nā mea maʻi, a hiki i kekahi manawa no ka piha hoolaia, ka hoʻohana 'ana o ka mālama' ana i nā māhele o nā cytostatics nō lākou ola expectancy hiki me ka nui loa hoʻonui.

He nui loa no ka poe me ka huikau Waldenstrom ka macroglobulinemia. Symptoms, ka prognosis o i ka loa loaa ole ia - amyloid ake a me nā konāhua, kahe ana ke koko a me ka pinepine? Acaeoey paraproteinemic coma. Inā manawa aole e kokua i ka poe e hoomanawanui i, he pahiki kiʻekiʻe o ka make.

Ka awelika ola expectancy no nā mea maʻi i ka maʻi mea e pili ana 4-5 makahiki. Ka wā pono unuhi aku la ia i ke kumumanao o ka iapaau ana e hoomahuahua ia i 9-12 makahiki.

'Ākena o ka maʻi

Ua haawiia i ka maʻi ia lŘlŘ kākaʻikahi, ia mea paakiki, e manao ia no ka hooulu ana o kekahi kanaka i kokoke loa i halawai me ia. Ua haawiia i ka mea mua hui 'ana me ka hoʻomanawanui i ka i nā kino, ia mea ma luna o ko lākou poʻohiwi, a me ka mea kuleana no ka hōʻoia' ana manaʻoi hoʻokoe 'i kēia, a diagnosed me Waldenstrom ka macroglobulinemia. He aha ka i kēia maʻi, e hoʻomanaʻoʻia,ʻaʻole i nā kauka, nae, i ka manaʻo i ka hoʻomanawanui i pololei haawi pathology, e lawe i ka mooolelo a pau kona lapaʻau hoike ana, e kekahi kino.

I ka wā e hiki mai, ua hoʻomanawanui, e alakaʻi hematologists, akā, i ka iniiaiieʻike,ʻanuʻu wale n? Ma luna o nā poʻohiwi o kauka nā keʻena lāʻau lapaʻau.

Late ka hōʻoiaʻana o ka maʻi hiki aku ai i ka loa hopena ia chemotherapy mea hou ka hoʻokō. I mea no ke aha la e ike oe e pili ana i keia ano maʻi a pau,ʻaʻole e hala ia, a mai i lawe mai ai i ka moku'āina o ka malama ole.