Macroglobulinemia Waldenstrom esithi: iimpawu, ebangela, abantu abane

Izifo igazi labakhonzi zinqabile, kodwa ziyingozi kakhulu abantu. Esinye isifo olunjalo Vandelstrema macroglobulinemia.

Ngaphezu kokuba esibonakala?

Phantsi kwesi sifo amayeza mihla kubhekiswa syndrome sofuzo hematological sikhatshwe ubukho macroglobulin kwegazi. Le iproteni ukhandwa umsebenzi B-lymphoid iiseli igazi monoclonal (B-lymphocyte).

Ngokuqhelekileyo, ezi seli banoxanduva Yindibanisela igazi-M globulin. Xa nawuphi na umsebenzi wamathambo (ngokufuthi kuphuhliso inkqubo thumba) kukho utshintsho acid acid ukubunjwa nolandelelwano zofuzo, okukhokelela kuyondelelaniswe macroglobulin, ngamanye ekuphuhliseni macroglobulinemia Waldenstrom kaThixo.

Esi sifo kakhulu inqabile, malunga-3 amatyala nganye abantu million. Yenzeka kakhulu kubantu abangaphezu kweminyaka yobudala engama-55. le ngezifo phantse lungenzeki kwiminyaka ukuya ku-40.

Njani, ke, ukubonakalisa sifo kwi kumaza inyama? Zeziphi izigqeba abachatshazelwa kwindawo yokuqala yaye ngenxa yoko apho?

Le Pathogenesis kwesifo

Xa intliziyo patogenza esi sifo ukwanda amabi weeseli plasma clone iiseli ekwaziyo zokuphuhlisa immunoglobulin M. Ezi seli angene umongo, isibindi, udakada, apho aqale ukwanda ngamandla kwaye cell macroglobulin izifo. Ngaleyo ndlela macroglobulinemia Waldenstrom kaThixo.

Ukwenyuka protein yayintsholongwane eyosulela egazini kukhokelela kuphuhliso viscosity yayo. Ngenxa isantya sokuqukuqela kwegazi ncitshiswa ngale ezityeni, nto leyo ekhokelela ekubeni kungekho kukhula wayo. Ukongeza, besebenzisa izinto coagulation plasma (ngenxa bokusonga okunqanda kubo macroglobulins). Ngenxa yale ephazamisa ukuyilwa ihlwili eziqhelekileyo, nto leyo ekugqibeleni kukhokelela ekwandeni ukopha.

Ngokweliso, xa kuvavanywa ngemicroscope, iiseli lomongo wethambo, ingabonwa awo apho "plazmatizirovannyh" lymphocyte, kukho inani elikhulu aqolileyo B-lymphocyte kunye secreting abalamli ukudumba weeseli imasti. Zonke ezi seli lubonisa ukuba kukho macroglobulinemia Waldenstrom.

iimpawu

Njani ngokwezonyango ingakrokreleka kwesi sifo?

Kwizigulane zokuqala Ndikhalaze kwizigulo nonspecific - ubuthathaka jikelele, icesina ephantsi-grade ixesha elide, ukubila, ukulahleka ngokobunzima ngaphandle kwesizathu.

Uphawu ephambili, ngokusekelwe apho Qalisa ukukrokra ukuba bavela Vandelstrema macroglobulinemia, Bakwanda wopha impumlo, iintsini, ngenwebu yangaphakathi. Kakhulu alufane waphawula ukugruzuka ongama nemivumbo.

Ekuhambeni kwexesha, ukuze aphuhlise hepatosplenomegaly kunye lymphadenopathy (iindawo nkovu idumbile). Kwakhona kuyenzeka uphuhliso kubunzima macroglobulinemia.

Xa ukusilela ukunika unyango ngexesha kukho umngcipheko wokuba isigulana isiqaqa, nkqu nokufa (kodwa zigulane ezikufutshane nokubhubha abakutshilo ayidli ngakuba nabungozi kakhulu kwinto bayahlupheka ukusuka macroglobulinemia Waldenstrom ngayo). Izinto ezibangela ukufa kwabo zibangelwa iingxaki zaphuhliswa.

iingxaki sifo

Kunjalo nzima esi sifo?

Ubukhulu becala inxaxheba kwinkqubo zophendlo lwezifo kwenkqubo sentliziyo, ezizezi izitya ezincinane - emithanjeni, arterioles kunye venules. Ngokunciphisa flow kwegazi waphawula uphuhliso retinopathy, nephropathy. Kidney umonakalo. Kulo mzekelo luphawulwa uphuhliso occlusion glomerular nemithambo kunye urolithiasis kunye yokulahlwa kwe urate.

Macroglobulinemia Waldenstrom esibonakala okukwazi eliphantsi kunye nokuzimanya usulelo olungenelelayo nzima kunye uxilongo esi sifo ngxabano.

Njengoko ukuqhubela phambili le nkqubo ingcinezelo wokuthi phantse zonke iintsholongwane ze hematopoiesis kunye nophuhliso pancytopenia. Amyloidosis esibonakala kuphuhliso amanxeba zophendlo lwezifo Ngokuka protein wesibindi kunye imithambo yegazi udakada, leyo mandundu ngakumbi ukuze umsebenzi wabo kakuhle.

ekuxilongweni

Yintoni metric kuya kukunceda uqonde ukuba bavela macroglobulinemia?

Okokuqala, simele sinikele ingqalelo ICBC. Izalathi ezingundoqo sifo iza kunyuka kwixesha erythrocyte rate neentlenge kwaye ukuyilwa "rouleaux" ezithile - ukuhlala ndawonye iiseli ezibomvu zegazi. Wbc ngokuqhelekileyo ibonisa inani elandileyo lymphocyte kuzo phaya kuhlalutyo yimbonakalo iifom leukocytes akuqolanga.

Ukuze uqinisekise uxilongo ngokuqhuba kumajoni omzimba. Emva ukuqhuba le sifundo akakwazi ukubona imali Yandisa immunoglobulin igazi M

immunoglobulins Monoclonal azimisele egazini emva nokongeza sera enombhalo.

Iimpawu ezongezelelweyo, kodwa hayi ezithile ziquka i isibindi eyandisiweyo kunye udakada, efumanise amyloid kwi biopsy kappillyarah kwizintso, kwakunye nasekunciphiseni eziliqela plasma coagulation (ngakumbi, into 8).

unyango

Kumanqanaba okuqala sifo, ngaphandle kokuba kukho ebonakalayo inxaxheba omzimba lwangaphakathi, unyango oluthile akukho kuboniswe. Amaxesha amaninzi, ezi zigulane bephantsi kweliso bezempilo udokotela wesithili.

Xa iimpawu lokuqala kwaye uqinisekise ukuba izifo - Waldenstrom macroglobulinemia, ukuqala unyango oluthile cytostatics. Kuba iziyobisi ukusebenzisa unyango ezifana "hlorbutin", "Cyclophosphamide". Zinelungelo isiphumo cytostatic ifihlakale umsebenzi Clone elibulalayo kakhulu B-lifotsitov.

Babekwe kule ndawo "hlorbutin" kuqala mg 6 imihla ngomlomo iiveki 3-4. Ukwandisa iidosi igcwele kunye nophuhliso umongo wethambo aplasia. Emva kokugqiba le khosi konyango emiselweyo amathamo nokugcinwa ichiza (4.2 mg) yonke eminye imihla.

Ukuphucula igazi rheological iparameters kusetyenziswa plasmapheresis. unyango plasmapheresis lwenziwa ukuba ukusetyenziswa kweziyobisi cytotoxic. Kuba inkqubo makasuswe loo eboniswe neelitha ezi-2 plasma kunye donor endaweni kwi inactivated yayo.

ukuvimbela

Ngenxa yokuba esi sifo ilifa, kunzima nangayiphi na indlela kuchaphazeleke inkqubela yawo, kuba sele ibhuqiwe phantsi zofuzo. Indlela kuphela kukuzama ukuphepha uphuhliso kwesifo ngexesha esi sifo kunye nesicwangciso kakuhle ezirhuqwa zonyango.

Ngqo kuchaphazela olo lunokuxhomekeka ubomi obusempilweni esi sifo, ukuyeka ukutshaya kunye notywala, umda etshaya kunye izitya zinongiwe.

Ekubeni isifo yemfuza, oko kubangele ukukhula kwayo kwezinye iimeko ezimbi - esebenza iidayi aniline, ipeyinti, ivanishi.

izifo rhoqo viral abakwaziyo ukutshintsha zofuzo zabantu. unyango kwangoko zezi zifo kuya kunceda ukuthintela utshintsho genes and iyaluthintela uphuhliso macroglobulinemia.

amanyathelo azizithintelo ekunokuthiwa ukuqhelanisa izigulane kunye iimpawu zesi sifo yaye kukhuthazwe imisebenzi yabo ukuthobela yokuphila esempilweni.

abantu abane

Yintoni ulindele kwizigulane abathe bona bazidina belungisa Waldenstrom macroglobulinemia? Abantu abane kwesi sifo kuxhomekeke ebukhulwini sifo esezikhona iingxaki kunye nonyango ngexesha elifanelekileyo.

Ukuba isifo sele sikwinqanaba lokuqala lophuhliso, kwiimeko ezininzi kunokwenzeka ukuthintela inkqubela yayo. izigulane ezifana nalo ithuba unyango epheleleyo, ukusetyenziswa amathamo nokugcinwa cytostatics namanje kwandisa ngokubonakalayo iminyaka yabo yokuphila.

Kuba kubi kakhulu abo macroglobulinemia nzima Waldenstrom kaThixo. Iimpawu, lo ALS apho Kuyimpoxo kakhulu - isibindi amyloid kunye nezintso, ukopha kunye nophuhliso rhoqo isiqaqa paraproteinemic. Ukuba ixesha swi nga pfuna izigulane ezinjalo, amathuba eliphezulu ukufa.

Umlinganiselo wobomi kwizigulane kunye sifo malunga 4-5 iminyaka. Xa umzobo ngokufanelekileyo necebo unyango ukwandisa ukuba kwiminyaka 9-12.

Ubungozi izifo

Njengoko esi sifo kwenzeka kunqabile, kunzima ukuba ukrokrela kuye kuphuhliso wendoda phantse ongazange wadibana naye. Njengoko qha yokuqala kunye zeetheraphisti bendawo yesigulane, oko emagxeni abo, kwaye linoxanduva lokuqinisekisa ukuba ngexesha kwathiwa ekurhaneleka kwaye kwafunyaniswa macroglobulinemia Waldenstrom kaThixo. Yintoni na le sifo, aziyi kukhunjulwa, asingabo bonke oogqirha, Noko ke, ukuba ucinga ukuba isigulana iye kunike- ngezifo, kuthathelwe ingqalelo yonke obubonakala lwezonyango, akufanele therapist.

Kwixa elizayo, lo uza kukhokela zegazi, kodwa uxilongo lokuqala lixhomekeke kuphela emagxeni kweekliniki oogqirha.

ukuchongwa emva kwexesha sifo kukhokelela imiphumo ebuhlungu xa ichemotherapy kungekho lusasebenza. Kungenxa yoko le nto kufuneka bazi ngale sifo bonke ukuba ukuphoswa kwaye musa kwimo yokungahoywa.