Erythremia - Iyini? eritremii esiteji

Erythremia - uhlobo zegazi ezibangelwa libhekene ayanda (overgrowth) zama-erythrocyte futhi ezinophawu ukwanda kwenani kwamanye amangqamuzana (leucocytes, nezinhlayiya zegazi). Ingabe okungamahlalakhona, isifo ngokuvamile ingozi kuyinto ngempela ezingavamile. Kuyinto Nokho ukonakala okungenzeka ingozi wegazi ebulalayo. Erythremia - isifo okuyinto iyagula ngabantu 4 abantu abayizigidi ezingu-100 ngonyaka ka. It akuxhomekile ubulili, kodwa ngokuvamile kutholakala abantu abadala nangaphezulu. Nakuba kubikwa lesi sifo ngesikhathi isencane, ngokuvamile abesifazane.

Erythremia (ikhodi ICD10 - S94.1) has a Yiqiniso ezingamahlalakhona.

Amangqamuzana egazi kanye indlela lesi sifo

indlela eritremii eba kanje.

Abomvu egazi ashaye - kwamangqamuzana abomvu engeziwe, futhi umsebenzi wakhe omkhulu - kuyinto ukuthwala umoya-mpilo ukuze wonke amaseli omzimba wethu. Basuke akhiqizwa umnkantsha, ubende kanye esibindini. Omnkantsha uku amathambo eside, amathambo, izimbambo, sternum ugebhezi amathambo. 96% walokho okubomvu egazi zigcwele elithwala umoya-mpilo, okuyinto lucwaningo umsebenzi zokuphefumula. umbala obomvu ibanika yensimbi. Amangqamuzana abomvu egazi akhiwa ngokusebenzisa ama-stem cell. Ukukwazi kwazo ehlukile - ikhono uhlukanise (ukukhiqiza kabusha), siguqulelwa yimuphi omunye cell.

Amathambo futhi ziphuzi futhi umnkantsha, okumelelwe okunamafutha izicubu. Uqala ukukhiqiza izakhi igazi kuphela ezimweni ezimbi kakhulu, lapho eminye imithombo hematopoietic hhayi imisebenzi yabo.

Erythremia (ICD-10 - International Ukwahlukanisa Diseases, lapho ikhodi kuleli kheli le-isifo 94,1 C) - a pathogenesis sifo azikaqondakali. Ngenxa yezizathu azinakho elicwengwé ngokugcwele ngodokotela, umzimba uqala ngomfutho ukukhiqiza amangqamuzana abomvu egazi angasasekelwa angena egazini, akhuphule umfutho viscosity, futhi baqala ukwakha amahlule. Ngokuhamba kwesikhathi, okwandisa hypoxia (i-oksijini yokulamba). Amaseli ungatholi kudla lokunemsoco lokwenele, ukungasebenzi kwenzeka ohlelweni jikelele zomzimba.

A amaqiniso ambalwa ezithakazelisayo mayelana eritremii

1. Erythremia - lesi sifo ingozi yazo zonke leukemias. Lokho isikhathi eside kangakanani yokwenziwa futhi kamuva kuphela kuholela ukuxakeka.
2. Kungenzeka asymptomatic futhi akusho ukumemezela ngokwawo iminyaka eminingi.
3. Naphezu ezingeni anda zegazi, iziguli othambekele ukopha kakhulu.
4. Erythremia inomlando umndeni, ngakho-ke uma umndeni has a abagulayo, ingozi ukubukeka ukukhuphuka isihlobo.

izizathu eritremii

Njengezinye izinhlobo wegazi, erythremia akukaqondwa ngokugcwele, kanye nezizathu ivela khona kungukuthi kwembulwe. Nokho, uzwakalise izinto cishe kunikela ukuvela yayo:

1. yofuzo.
2. ezinobuthi abanjwe emzimbeni.
3. Ionizing emisebeni.

yofuzo

Nokho, akwaziwa kahle hle ukuthi yiziphi zofuzo kubangele eritremii ukubukeka, lwathola ukuthi isifo umndeni ivela kaningi ngokwanele ezizukulwaneni esilandelayo. Amathuba asathuthuka lesi sifo kukhuphuke ngo izikhathi eziningana, uma umuntu ukuhlupheka:

• -Down syndrome (kokunikezwa kwe-ebusweni nasentanyeni, ukubambezeleka zentuthuko);
• Klinefelter Syndrome (kungenzeka sibalo ongalingani futhi ukulibaziseka kwamabanga);
• Bloom Syndrome (ukuphakama ongaphakeme, lesikhumba ebusweni ongalingani ukuthuthukiswa nezici ezithile zalo ezifweni oncological);
• -Marfan syndrome (iziyaluyalu ezicutshini).

Nezici ezithile ukuze eritremii wachaza iqiniso lokuthi ngezakhi zofuzo imishini yeselula (kuhlanganise igazi iyunithi) ingahlalisekanga, ngakho umuntu eba ukungenwa umthelela zangaphandle engakhi - ubuthi, emisebeni.

ngemisebe

Ngisho-X-ray kanye gamma ray kuthiwa ngokwengxenye amuncwa wumzimba, ezithinta amaseli zofuzo. Bangafi noma ishintshe.

Nokho, emisebeni enamandla kakhulu enemisebe wathola abantu abaphatha umdlavuza ngamakhemikhali okwamenza lesi sifo, kanye nalabo ababe kube yiyo eshayeka kanzima izitshalo amandla noma amabhomu e-athomu.

ezinobuthi

Lapho udle, ziyakwazi okubangela kwezakhi zamangqamuzana. Laba basizi abizwa ngokuthi mutagens zamakhemikhali. Ngemva izifundo eziningi, ososayensi baye bathola ukuthi iziguli eritremii uxhumane nala makhemikhali ngaphambi ukuthuthukiswa lesi sifo. Lezi izinto zihlanganisa:

• benzene (atholakala Ukwakheka zikaphethiloli kumakhemikhali awuketshezi eziningi zamakhemikhali);
• ejenti elwa namagciwane (ngokukhethekile, "Chloramphenicol");
• ejenti cytostatic (anti-isimila).

izigaba Eritremii futhi izimpawu zabo

Erythremia has izinyathelo ezilandelayo: kokuqala, zenzeka futhi ukuphela. Ngamunye wabo inezimpawu lalo. Esigabeni sokuqala amashumi ngaphandle kokukubonisa iyiphi izimpawu zokugula okungathí sina. izimpawu Minor iziguli ngokuvamile sola ngakolunye, izifo ezincane. Phela, ngisho odokotela bavame bamshaya indiva ngokwanele ukuba abangaphelele kwegazi.

Uma kukhona erythremia eyinhloko, ukuhlolwa kwegazi babe ngeziphambeko ngokulinganisela kusukela kuyinsakavukela.

Esigabeni sokuqala nakho libhekene ukutubeka komzimba, isiyezi, tinnitus. Isiguli silala kahle, ukuzwela ukushisa lapho kubanda e emikhawulweni kunezindatshana ukuvuvukala ezandleni nasezinyaweni. A ukwehla ukusebenza kwengqondo. Ayikho ukubonakaliswa kwangaphandle isifo okwamanje. Ikhanda elibuhlungu akubhekwa uphawu ethize lesi sifo, kodwa kwenzeka lapho besanda kuqala ngenxa kahle kwegazi ebuchosheni. Ngenxa yalesi sizathu, encishisiwe umbono, ukunakekela kanye nezobunhloli. Lapho sitholakele "erythremia" Izimpawu zesifo esigabeni sesibili anezici zabo - nokopha izinsini, iba hematoma encane. On umlenze ephansi kungase kubonakale amnyama (izimpawu thrombosis) ngisho izilonda. Khulisa izitho zomzimba ukukhiqiza amangqamuzana egazi abomvu - ubende kanye esibindini. Ngenxa ukuthuthukiswa lesi sifo zingavuvukalanga zamanzi.

Eritremii isigaba sesibili izothatha iminyaka engu-10. Isisindo is kakhulu ziyancipha. Isikhumba uthola cherry ithoni (ngokuvamile kwezandla nezinyawo), ulwanga ezithambile kushintsha umbala, okuqinile - igcina nombala ubudala. Ukulunywa ukhathazwa isiguli okuyinto iyakhula ngemva eyigeza emanzini afudumele noma kushisa. Umzimba uba umthambo evuvukele kusukela overabundance wegazi, ikakhulukazi entanyeni. Eyes kwakubonakala bloodshot, ngoba erythremia, izimpawu zazo zisobala okwanele esigabeni sesibili, ikhuthaza igazi ligeleze izitsha iso.

Ngenxa abampofu kwegazi e-capillary wabonakaliswa ubuhlungu futhi ukushiswa ukuzwa kule iminwe yakhe nezinzwani zakhe. Ezimweni eziphambili sobala tsiatonicheskie izindawo kuwo.

Kukhona amathambo ubuhlungu epigastric. ubuhlungu bamalunga ezihambisana nemvelo gouty ngokweqile uric acid. Ngokuvamile, umsebenzi izinso iphazamisekile, ngokuvamile kutholakala ukuthi uphethwe yini pyelonephritis futhi wathola uratovye ezinso.

Isimiso sezinzwa uyahlabeka. Isiguli sezinzwa, isimo sakhe ingahlalisekanga, libhekene tearfulness nezinguquko njalo.

viscosity ukwanda igazi, eliphumela ukubukeka amahlule ezindaweni ezihlukahlukene semithambo. Futhi kukhona ingozi yokuba ukuthuthukiswa varicose emithanjeni.

Isexwayiso! Iziguli nabo bangase bahlupheke ngenxa umfutho wegazi ophakeme, futhi kuvulwa ulcer duodenum. Lokhu kungenxa ukwehla umzimba ozivikela futhi ukwandisa isibalo Helicobacter pylori - lokhu igciwane kubangela izilonda.

Eritremii Isigaba sesithathu libhekene pallor kwesikhumba, faintness njalo, ubuthakathaka kanye namandla. Kubhekiwe ukopha okungapheli phakathi ukulimala okuncane, anaemia aplastic ngenxa aphansi e-hemoglobin amazinga egazini.

Esesithathu eritremii esiteji: Izimpawu kanye Izinkinga

Erythremia - isifo ngegazi, esigabeni sesithathu iba nolaka. Kulesi sigaba, amaseli umnkantsha ibhekane cystic. Akakwazi ukukhiqiza amangqamuzana egazi abomvu, ngakho imithetho kwamangqamuzana egazi awela, ngezinye izikhathi izinkomba esibucayi. Ebuchosheni, kukhona foci of ukuthambisa, isibindi cystic iqala. Esinyeni nenyongo awugqinsi viscous Bile futhi sombala amatshe. Umphumela kuba isifo sokusha futhi occlusion semithambo.

thrombus Ngaphambili, senyuselwa kungaba abe imbangela yokufa kweziguli erythema. isitsha izindonga iyashintsha, kukhona ukuvaleka emithanjeni ebuchosheni, ubende, inhliziyo nasemilenzeni. Kuphakama isifo occlusive - ukuvaleka imithambo imilenze nge ingozi uyisifushaniso egcwele.

ukulimala kwezinso. Ngenxa ukukhula iziguli uric acid uhlushwa ubuhlungu bamalunga uhlamvu gouty.

Isexwayiso! Lapho imilenze eritremii nezingalo ngokuvamile ukushintsha umbala. Isiguli uthambekela ukucinana kwemigudu yokuphefumula kanye imikhuhlane.

lesi sifo

Erythremia - isifo kancane. Lesi sifo angabhekana siqu kancane yayo, ukuqala uhlamvu okuqhubekayo futhi avulekile. Iziguli ngokuvamile uphile iminyaka ngeminyaka ngaphandle kokunaka izimpawu ezincane. Nokho, ezimweni ezibucayi ngaphezulu ngoba amahlule ukufa angenzeka hlangana iminyaka 4-5.

Kanye eritremii anda ubende. ifomu ezisemajukujukwini isifo sokusha lesion futhi diencephalon. Phakathi kungenzeka izifo umzimba uxabane nawo, kanye nezinkinga ezithathelwanayo abathintekayo, iziguli kaningi awukwazi ukubekezelela imithi ethile iqembu wahlushwa urticaria nezinye izifo zesikhumba. Inkambo isifo nzima izimo co-okwenzeka, uphethwe Ngokuvamile kwenziwa ngemva asebekhulile.

myelofibrosis Posteritremichesky - kabusha inkambo ingozi lokugula e ebulalayo. Isimo kuyinto engokwemvelo ezigulini ezisekhona kuze kube kulesi sikhathi. Kulokhu, odokotela bayavuma ukuthi erythremia atholwe isimila uhlamvu.

Kubaluleke kakhulu ukuba kahle ukuxilonga erythremia futhi ukwelashwa okufanele. Sifo kuhlanganisa eziningi izifundo.

Ukuhlaziywa kwegazi eritremii

Lapho sitholakele "erythremia" Amapharamitha ukuhlolwa igazi okuyizinto ezibalulekile for uphethwe, kwenziwa kwasekuqaleni. Ngokuyinhloko lokhu kuwuvivinyo igazi ezivamile. Kuyinto okungavamile kuye ekubeni ngowokuqala alamu insimbi. It isibalo kwamangqamuzana egazi abomvu. Ekuqaleni, akusiyo ezibucayi Nokho, ukuthuthukiswa sifo echuma kakhulu, futhi uwela esiteji zokugcina. I evamile erythrocyte ezingeni egazini abesifazane ivela 3.5 4.7, futhi amadoda - 4 kuya 5.

Uma sisheshe erythremia, ukuhlolwa kwegazi izibalo ebonisa ukwenyuka abomvu egazi, lithini ukwanda okuhambisanayo elithwala umoya-mpilo. Hematocrit ekhombisa ikhono igazi ukuze athwala umoya-mpilo, lakhuphuka lafika ku-60-80%. Umbala Inkomba engashintshwa, kodwa esigabeni sesithathu kungaba yini - evamile, anda noma encishisiwe. Ama-platelet I futhi amangqamuzana amhlophe egazi anda. Amangqamuzana amhlophe egazi sikhule izikhathi eziningana, futhi ngezinye izikhathi ezingaphezu. Ekwakhiweni eosinophils (kwesinye isikhathi basophils) kwandisa. Ngokuzimisela anda platelet. Erythrocyte sedimentation izinga - hhayi ngaphezulu kuka-2 mm / ihora.

Maye, ephelele kakhulu segazi, nakuba kubalulekile ukuze kuhlonzwe kokuqala, ukwaziswa okuncane, bese kuphela ngesisekelo ekuxilonga ungacimi.

Olunye ucwaningo ngezinye eritremii

• ukuhlaziywa kwamakhemikhali kwegazi. Umgomo wayo lomkhulu - ukunquma inani zensimbi egazini kanye nezinga umsebenzi ukuhlolwa isibindi - AST no-ALT. Bayakhululwa kusukela kwesibindi ekubhubhiseni amaseli ayo. Ukunqunywa inani bilirubin kubonisa kobukhulu inqubo erythrocyte ekubhujisweni.
• Omnkantsha wokubhoboza. Ukuhlaziywa esithathwa Inalidi ufakwa isikhumba kuya periosteum. Indlela ibonisa isimo amaseli hematopoietic umnkantsha. Ucwaningo lubonisa inani lamaseli e-umnkantsha, khona amangqamuzana omdlavuza futhi cystic (ukukhula sezicubu zezinzwa).
• Lapho sitholakele "erythremia okungamahlalakhona" futhi wenze nakomaka laboratory ultrasound besisu. Ultrasound lembula izitho eminyene, isibindi lakhulisiwe futhi ubende, kanye ekhukhwini cystic kuzo.
• Doppler kubonisa ukugeleza kwegazi velocity kuyabaveza amahlule.

ukwelashwa eritremii

Kufanele kuqashelwe ukuthi erythremia ngokuvamile sanda kancane kancane futhi Yiqiniso kungavamile ukuba ingozi. Okokuqala, odokotela belulekwa ukuba wenze izinguquko yokuphila - ngaphezulu ukuba ngaphandle, ukuhamba, ukuze uthole imizwelo eyakhayo (ngezinye izikhathi endorfinoterapiya ifinyelela Imiphumela omkhulu). Ukudla okune-ingxenye semali zensimbi Vitamin C, kufanele indiva.

Ngo esigabeni sokuqala lesi sifo injongo eyinhloko uphethwe "erythremia" - nemingcele igazi ukunciphisa i-hemoglobin evamile - kuze kufinyelele 150-160, futhi hematocrit - ukuze 45-46. Kubalulekile futhi ukuba negate yezinkinga okubangelwa isifo - iziyaluyalu kwegazi, ubuhlungu eminweni, njll ...

Kujwayeleke hematocrit nge-hemoglobin kusiza ukopha, lokukhulunywa ngako kulendzaba isetshenziswa kuze kube yilolu suku. Nokho, le nqubo kwenziwa kohlaka esiphuthumayo, ngoba kukhuthaza umnkantsha amathambo thrombopoiesis umsebenzi (platelet kumiswa inqubo). Kukhona inqubo okuthiwa eritrotsitaferez, okuyinto ukuze ahlanze igazi elibomvu egazi. Kulokhu, oketshezini olungenambala lwegazi uhlala.

imithi

Cytostatics - hlobo antitumor izidakamizwa ezisetshenziswa ngaso yezinkinga eritremii - izilonda, thrombosis, cerebral kwegazi iziyaluyalu zingaveli nesigaba lesi sifo yesibili. "Mielosan", "busulfan", hydroxyurea, "Imifos" phosphorus enemisebe. Lesi sakamuva kubhekwa ngempumelelo ngenxa yokuthi zanqwabelana amathambo futhi kuvimbela ekusebenzeni kobuchopho.

Ama-steroid engabelwe anaemia hemolytic Lokudabuka sokuhlasela kwamasosha omzimba. "Prednisolone" othandwa kakhulu. Uma impatho akukuniki imiphumela efanele, linqume ukuhlinzwa ukhishwe ubende.

Ukuze ugweme yensimbi ukuntuleka kwamavithamini, ukungezwani yensimbi Izithako ezingahle zinqunywe - "gemofer", "Totem", "Sorbifer".

Amaqembu elandelayo izidakamizwa kuyabekwa njengoba kudingeka:

1. Antihistamine.
2. Ukwehlisa blood pressure.
3. Anticoagulant (igazi thinning).
4. Hepatoprotectors.

Erythremia, impatho ngokuvamile kwenziwe esibhedlela - isifo esiyingozi esibangela eziningi izinkinga. Kubalulekile ukukhomba kusenesikhathi bese uqale ukwelashwa kwakhe.