Eritrea yintoni? Izigaba ze-erythremia

I-Erythremia yenye yeentlobo zezifo zegazi ezibonakalisa ukwanda (ukwanda) kwe-erythrocytes kunye nokunyuka okubonakalayo kwinani lezinye iiseli (leukocytes, platelets). Kuyinto engapheliyo, ihlala isifo, isifo esingafanekiyo. Nangona kunjalo, kunokwenzeka ukuba i-leukemia ebenzileyo ibe yinto enobungozi. I-Erytremia yisifo esichaphazela abantu aba-4 ngonyaka ukusuka kubantu abayizigidi ezili-100 ngonyaka. Akuxhomekeke kwisini, kodwa ngokuqhelekileyo ixilongwa kubantu abadala nakhulileyo. Nangona iziganeko ezibhalisiweyo zesifo kwaye zisencinane, ngokuqhelekileyo ngabafazi.

I-Erythremia (ikhowudi ICD-10 - C94.1) inenkqubo engapheliyo.

Iiseli zegazi neendlela zesifo

Indlela yo-erythremia ikhula ngale ndlela ilandelayo.

I-Erythrocytes ngamaseli obomvu, umsebenzi oyintloko wokudluliselwa kwe-oksijini kuzo zonke iiseli zomzimba wethu. Ziveliswa kwintolongo yethambo, ipeni kunye nesibindi. Umnkantso we-Bone ufunyanwa ngamathambo abulalayo, ama-vertebrae, iimbambo, amathambo kunye ne-sternum. Kwi-96%, iiseli ezibomvu zegazi zizaliswa nge-hemoglobin, eyenza umsebenzi wokuphefumula. Umbala obomvu unikezelwa ngetsimbi. I-Erythrocytes yenziwe yileli-stem cell. Amandla abo ahlukeneyo kukukwazi ukwabelana (ukuphindaphinda), ukuguqula nakweyiphi enye iseli.

Emathanjeni, kukho umnatha wethambo ophuzi, omelelwe ngamathambo e-adipose. Uqala ukuvelisa izinto zegazi kuphela phantsi kweemeko ezimbi kakhulu, xa imithombo eseleyo ye-hematopoies ingenzi imisebenzi yayo.

I-Erythremia (i-ICD-10 ikwahlukana ngamazwe ngamazwe apho le ngxaki yanikwa ikhowudi C 94.1) isifo esiyi-pathogenesis esingafundiwe. Ngenxa yezizathu ezingaqondakali ngokupheleleyo oogqirha, umzimba uqala ngokunyanzela ukuvelisa iiseli ezibomvu zegazi, eziyeka ukulungelelanisa igazi, i-viscosity yegazi iyakwanda, kunye neengcezu ziqala ukwenza. Emva kwexesha, ukwanda kwe-hypoxia (i-oxygen starvation). Iiseli azifumani ukondla olufanelekileyo, kukho ukungaphumeleli kwinkqubo yonke yomzimba.

Ezinye izinto ezithandekayo malunga ne-erythremia

1. I-Erytremia yinto yesifo esinobuhlungu kakhulu yeeukemi. Oko kukuthi, ixesha elide liziphatha ngokungenakuqhelana kwaye likhokelela kwiinkathazo kunokuba ziphelile.
2. Ingaba yinto engabonakaliyo kwaye ungazibikeli iminyaka emininzi.
3. Nangona ukwandiswa kweeplatelet, izigulane zixhomekeke ekuphumeni kwegazi.
4. U-Erytremy unentsapho, ngoko ke ukuba kukho isigulane kwintsapho, umngcipheko wokubonakala kwayo kwesinye sezihlobo zanda.

Izizathu zeErythema

Njengezinye iintlobo ze-leukemia, i-erythremia ingazange ifundwe ngokupheleleyo, kwaye izizathu zokubonakala kwazo azibonakalwanga. Nangona kunjalo, izinto ezikhoyo zichazwe:

1. I-Genetic predisposition.
2. Izinto ezinobungozi emzimbeni.
3. Ionizing radiation.

I-Genetic predisposition

Okwangoku, awaziwa ukuba yiyiphi iinguqu zokuguqula izakhi kubangela ukubonakala kwe-erythremia, kodwa kusekwa ukuba intsapho iphindaphindiweyo kwizizukulwana ezizayo. Ubunokwenzeka bokuphuhlisa eso sifo bunyuselwa ngamaxesha amaninzi, ukuba umntu uhlaselwa:

• I-Down's syndrome (ukuphulwa komfanekiso wobuso kunye nentamo, ukulahla ekuphuhliseni);
• Isifo se-Klinefelter (umlinganiselo ongafaniyo kwaye unakho ukuphuhliswa kokuphuhliswa);
• I-Bloom's syndrome (ukukhula okuphantsi, ukuguqulwa kwesigqabiso ebusweni kunye nophuhliso lwayo olungenakuphikiswa, ukulungelelaniswa kwezifo ze-oncological);
• I-Marfan syndrome (ukuphazanyiswa kophuhliso lwezicubu ezinxibelelwano).

Ukubonakalisa i-erythremia kubangelwa ukuba izixhobo zeselfini zezofuzo (kubandakanya i-apparatus yegazi) zizinzile, ngoko-ke umntu uya kuba neempembelelo zangaphandle ezibi - i-toxins, i-radiation.

I midiyo

Ngaphandle kwe-X-rays kunye nemigqa ye-gamma ixutywe ngumzimba, ichaphazela iiseli zomzimba. Baya kufa okanye batshintshe.

Nangona kunjalo, imisebe enamandla kunazo zonke ifunyenwe ngabantu abaphatha umhlaza ngekhemotherapy, kunye nalabo babesesigxina sezityalo okanye iibhomu ze-athomu.

Izinto ezinobuthi

Xa utshisiwe, banako ukudala ukuguqulwa kwamaseli e-genetic. Ezi zinto zibizwa ngokuba yi-chemical mutagens. Emva kwezifundo ezininzi, izazinzulu zathola ukuba izigulane ezine-erythremia zidibanisene nalezi zinto ngaphambi kokuba isifo siphuhliswe. Ezi zinto ziquka:

• I-Benzene (efumaneka kwi-petroli kunye neengcombolo ezininzi zamakhemikhali);
• Izidakamizwa ze-antibacterial (ngakumbi, "Levomycetin");
• Iziyobisi ze-Cytostatic (iziyobisi ezichasayo).

Amanqanaba e-erythremia kunye neempawu zabo

Isigaba se-Erythremia sinoku: okuqala, ukwandiswa kunye nesigxina. Ngamnye wabo unempawu zakhe. Isigaba sokuqala sinokuhlala iminyaka emininzi ngaphandle kokubonisa nayiphi na impawu ezinzulu. Iimpawu ezincinci zidla ngokubhekiselele kwezinye izifo ezincinci. Emva kwakho konke, kwanokuba oogqirha badla ngokuhlawula ngenxa yokuhlaziywa kwegazi okungelona ndawo.

Ukuba kukho i-erythremia yokuqala, iimvavanyo zegazi zineempembelelo ngokuthe ngqo kwi-standard.

Isigaba sokuqala sichazwe ngokukhathala ngokukhawuleza, ukuthuthumela, isandi ezindlebeni. Isigulane asilali kakuhle, siziva sishushu kwiimilenze, kukho ukuvuvukala kwezandla kunye neenyawo. Kukho ukwehla kwentsebenzo yengqondo. Akukho zibonakaliso zangaphandle zesifo okwamanje. Intloko ayiqwalaselwe uphawu oluthile lwesifo, kodwa lwenzeka kwinqanaba lokuqala ngenxa yokujikeleza kwegazi ephosakeleyo kwingqondo. Ngesizathu esifanayo, umbono, ingqalelo kunye nobuchule kuncitshiswa. Xa ukuxilongwa kweempawu ze "erythremia" zesifo kwisigaba sesibini kunempawu zaso - iimpawu zesisu, i-hemomom ezincinane ziyabonakala. Emlenzeni ongezantsi ungabonakala amabala amnyama (iimpawu ze-thrombosis) kunye nezilonda ze-trophic. Amalungu apho i-erythrocytes eyakhiwa ikhulisiwe, - ipeni nesibindi. Ngenxa yokuphuhliswa kwesi sifo, i-lymph nodes ikhula.

Isigaba sesibini se-erythremia sinokuhlala malunga neminyaka eyi-10. Ubunzima buyancitshiswa. Ikhumba lifumana ithoni ye-cherry (ngokuphindaphindiweyo iingalo nemilenze), isalathisi esithambileyo sitshintsha umbala, isalathisi esinzima sigcina umthunzi wayo wangaphambili. Isigulane siphazamiseka ngesikhumba, esomeleleyo emva kokuhlamba emanzini atshisayo okanye ashushu. Emzimbeni, iimvumba ezivuvukayo zibonakala zivela kwigazi elingakumbi, ngakumbi entanyeni. Amehlo afana negazi, kuba i-erythremia, iimpawu zazo ezibonakalayo ngokufanelekileyo kwisigaba sesibini, kuncedisa ekuqhubeni kwegazi kwiinqanawa zamehlo.

Ngenxa yokusasazeka kwamathambo kwi-capillaries, intlungu kunye nokuvutha komlilo kwiminwe nasezinzwaneni zibonakala. Amatyala anganakukhatyathwa, amabala e-cyanotic aya kubonakala.

Iintlungu zesifo se-Bone ne-epigastric zenzeka. Intlungu ye-arthritic joint idibene ne-uric acid excess. Ngokuqhelekileyo, umsebenzi wezintso uphazamiseka, i-pyelonephritis ixilongwa rhoqo kwaye igaye amatye kwiintso zifunyenwe.

Inkqubo yesifo iyabuhlungu. Isigulane sinesifo, isimo sakhe sengqondo asiqinisekanga, sichaphazela ukukhalazela kwaye kaninzi sitshintsha.

I-viscosity yegazi iphakama, ekhokelela ekubonakaleni kwe-thrombi kwiindawo ezahlukeneyo zeesistim. Kukho umngcipheko wokwakha imithanjeni ye-varicose.

Qaphela! Izigulana nazo zinokuxhatshazwa yingozi yengozi kunye nokufunyanwa kwezilonda kwi-duodenum. Oku kubangelwa ukwehla kwimizimba yomzimba kunye nokwanda kwenani le-Helicobacter pylori - yile bhaktiriya ebangela isilonda.

Isigaba sesithathu se-erythremia sibonakaliswa yintsiba yesikhumba, rhoqo ukuphelelwa amandla, ubuthathaka kunye nokunyaniseka. Kukhona ukuphuma kwegazi ngexesha elide ngexesha lokulimala kakhulu, i-aplastic anemia ngenxa yokunciphisa izinga le-hemoglobin egazini.

Isigaba sesithathu se-erythremia: iimpawu kunye neengxaki

I-Erythremia isifo segazi esinobudlova kwisigaba sesithathu. Kule nqanaba, iiselomnyo zomnatha ziphantsi kwe-fibrosis. Akakwazi ukuvelisa iiseli ezibomvu zegazi, ngoko imimiselo yamaseli egazi ihla, ngamanye amaxesha kumanqanaba amanqanaba. Kwingqondo, i-foci ye-softening ivela, i-fover fibrosis iqala. Kwi-gallbladder yinto enobuninzi, i-viscous bile ne-pigmented stone. Isiphumo salokhu yi-cirrhosis yesibindi kunye nokukhutshwa kwemithambo yegazi.

Ngaphambili, kwakusungulwe ukwakheka kwe-thrombus eyabangela ukufa kwezigulane ezine-erythremia. Iindonga zeempahla zixhomekeke kutshintsho, kukho ukucinywa kweemvini kwiinqanawa zengqondo, ipeni, intliziyo nemilenze. Kukho ukuphela kwe-endarteritis-occlusion yeempahla zemilenze kunye nomngcipheko we-constriction yazo epheleleyo.

Izintso zichaphazelekayo. Ngenxa yokukhula kwe-uric acid, izigulane zineentlungu zesifo se-arthritic somntu onobunzima.

Qaphela! Nge-erythremia, imilenze kunye neengalo zihlala ziguqula umthunzi wazo. Isigulane sithandana ne-bronchitis kunye namaqanda.

Inkambo yesi sifo

I-Erytremia yisifo esicothayo. Esi sifo sifikela ngokwazo ngokukhawuleza, ukuqala kwayo kuhamba phambili kwaye kunomntu oqhetsebayo. Ngezinye izigulane baphila iminyaka emininzi, bengayithobeli ingqalelo iimpawu ezincinane. Nangona kunjalo, kwiimeko ezinzulu, ngenxa ye-thrombi, ukufa kungenzeka kwiminyaka engama-4-5.

Kanye ne-erythremia, i-spleen iyanda. Gxotha ifom nge-cirrhosis yesibindi kwaye unqobe ingqondo ephakathi. Ngethuba lokugula, iingxaki ezichaphazelekayo nezichaphazelekayo zingabandakanyeka, ngamanye amaxesha izigulane azikhuthazeli amaqela atywala, aphethwe yimifuno nezinye izifo zesikhumba. Inkambo yesi sifo iyanzima ngeemeko ezixubileyo, kuba ukuxilongwa kudlalwa kubalupheleyo.

I-post-arrhymic myelofibrosis yintsholongwane yesifo esivela kwinkambo yokuzibulala ibe yingozi. Imeko yendalo kwizigulane ezasinda kulo xesha. Kule meko, oogqirha bayavuma ukuba i-erythremia ifumane ubunjani be-neoplastic.

Kubaluleke kakhulu ukufumanisa kakuhle i-erythremia kwaye unika unyango olufanelekileyo. Ukuxilongwa kuqukwa kwimiba yezifundo.

Uvavanyo lwegazi lwe-erythremia

Ngokuxilongwa "kwe-erythremia", ukuhlolwa kwegazi, izibonakaliso ezibaluleke kakhulu ukuxilongwa, zenziwe kuqala. Okokuqala, oku kuhlolwa kwegazi jikelele. Yiyo ukuphambuka okuvela kwinto evamile kuye eyakuba yintsimbi yokuqala ye-alamu. Ukwandisa inani lamaseli obomvu. Ekuqaleni, akunjalo, kodwa ngokuphuhliswa kwesi sifo kwandisa ngakumbi, kwaye kwinqanaba lokugqibela liyawa. Ngokuqhelekileyo, izinga le-erythrocytes egazini labasetyhini liphakathi ko-3.5 no-4.7, kwaye kumadoda-ukusuka ku-4 ukuya ku-5.

Ukuba i-erythremia ifunyaniswa, ukuhlolwa kwegazi, okubonisa ukwanda kwe-erythrocytes, kukhombisa ukwanda okwenziwe kwi-hemoglobin. I-Hematocrit, ebonisa ubuchule begazi ukuthwala i-oksijeni, yanda ukuya kuma-60-80%. Isibonakaliso sombala asitshintshi, kodwa kwisigaba sesithathu sinokuthi sinjani - esivamile, ephakamileyo okanye ephantsi. I-thrombocytes kunye neukocycytes zonyuka. I-leukocytes ikhula kaninzi, kwaye ngamanye amaxesha nangakumbi. Ukuveliswa kwama-eosinophil (ngamanye amaxesha kunye kunye ne-basophil) kuphuculwe. Iiplatelets eziphakamileyo. Isantya se-erythrocyte sedimentation asikho ngaphezu kwe-2 mm / hr.

Hayi, ukuhlalutya ngokubanzi kwegazi, nangona kubalulekile ekuxilongweni kokuqala, akunakulwazi oluncinane, kwaye kuphela kwisiseko sayo ukuxilongwa okwenziwe.

Ezinye izifundo kunye ne-erythremia

• Hlalutyo lwegazi. Injongo yaso ephambili - ukujonga inani lentsimbi egazini kunye nomgangatho weemvavanyo zesibindi - i-AST ne-ALT. Ziyakhululwa kwisibindi xa iiseli zitshabalaliswa. Ukumisela kwexabiso le-bilirubin kubonisa ubunzima benkqubo yokubhujiswa kwe-erythrocyte.
• Ukugqithwa kwethambo lomongo. Uhlalutyo lwenziwa ngenaliti, ejoyiweyo kwesikhumba, ukuya kwi-periosteum. Le ndlela ibonisa isimo seeseli ze-hematopoietic emnxeni wethambo. Uphononongo lubonisa inani lamaseli emnzini wethambo, ubukho beeseli zomhlaza kunye ne-fibrosis (ukwandisa kwezicubu ezinxibelelwano).
• Xa ukuxilongwa "kwe-erythremia engapheliyo" kwenza amanqaku ebhoratriki kunye ne-ultrasound yesisu esiswini. I-Ultrasound inokubona ukugqithiswa kwamalungu kunye negazi, ukwandiswa kwesibindi kunye ne-spleen, kunye ne-foci ye-fibrosis kuwo.
• I-Dopplerography ibonisa ijubane lokuhamba kwegazi kwaye inceda ukuchonga i-thrombi.

Unyango lwe-erythremia

Kufuneka kuqatshelwe ukuba i-erythremia isoloko iqhubela phambili kwaye ihamba kakuhle. Okokuqala kwaye phambili, oogqirha bacebisa ukuba batshintshe indlela yobomi-ukuba baphume ngaphandle, bahambe, bafumane iimvakalelo ezintle (i-endorphin yonyango ngamanye amaxesha iphumelela kwimiphumo emangalisayo). Imveliso equlethe ininzi yesinyithi kunye ne-vitamin C kufuneka ikhutshwe.

Kwinqanaba lokuqala lesi sifo, injongo ephambili yokuxilongwa kwe "erythremia" kukunciphisa amanqanaba egazi ngokuqhelekileyo: i-hemoglobin ukuya kwi-150-160, kunye ne-hematocrit ukuya kwi-45-46. Kwakhona kubalulekile ukuphazamisa iingxaki ezibangelwa zizifo-izijikelezo ezijikelezayo, intlungu eminwe, njl njl.

Ukuqhelanisa i-hematocrit ne-hemoglobin inceda ukuphuma kwegazi, oku kuthethwa ngawo kulolu suku. Nangona kunjalo, le nqubo yenziwa njengenxalenye yokhathalelo lwengxamiseko, kuba ivuselela umongo wethambo kunye nomsebenzi we-thrombopoiesis (inkqubo yokwenza iplatelet). Kukho inkqubo ebizwa ngokuthi i-erythrocytapresis, equka ekuhlambuleni igazi kwiiseli ezibomvu zegazi. Kule meko, i-plasma yegazi ihlala.

Amachiza

Izidakamizwa ze-cytotoxic zilungelelwano lwezinto ezichasene nazo ezisetyenziselwa iingxaki ze-erythremia-ulcers, i-thromboses, ukuphazamiseka kwentsholongwane yengqondo eyenzeka kwisigaba sesibini sesi sifo. "Myelosan", "Busulfan", i-hydroxyurea, "i-Imifos", i-phosphorus ene-radioactive. Olu hlobo lokugqibela lubonwa ngempumelelo ngenxa yokuba luqokelela amathambo kwaye luphazamise umsebenzi wengqondo.

I-Glucocorticosteroids imiselwe i-hemolytic anemia ye-auto-immune genesis. Eyona nto ithandwayo yiPrednisolone. Ukuba unyango aluniki iziphumo ezifanelekileyo, kusetyenziswe umsebenzi ukuba ususe ipeni.

Ukuze ugweme ukusilela kwintsimbi, ukulungiselela i-iron-containing containing "Hemofer", "Totema", "Sorbifer" inokumiselwa.

Kwakhona, ngokufanelekileyo, amaqela alandelayo asebenzisa iziyobisi ayalelwe:

1. Antihistamines.
2. Ukunciphisa uxinzelelo lwegazi.
3. Ama-Anticoagulants (igazi elincinci).
4. Hepatoprotectors.

I-Erythma, esoloko iphathwe esibhedlele, isifo esibalulekileyo esibangela iingxaki ezininzi. Kubalulekile ukuyijonga ngokukhawuleza kwaye uqale unyango.