I-Myeloproliferative neoplasms (MPN), eyayibizwa ngokuba yi-myeloproliferative disorders, i-group of disorders ibonakalisa ukwanda kwe-one okanye ngaphezulu kweeseli zegazi (amangqamuzana egazi ezimhlophe, iiseli ezibomvu zegazi kunye / okanye iplatelets). Nangona unako ukudibanisa igama le- neoplasm nomhlaza, akusiyo loo nto ecacileyo.
I-Neoplasm ichazwa njengokwanda okungavamile kwezicubu ezibangelwa ukuguqulwa kwaye zingachazwa njengezintlu (ezingekho komhlaza), zangaphambi komhlaza, okanye zizifo zomhlaza (ezibi kakhulu).
Xa ixilongwa, ezininzi izilwanyana ezihamba nge-myeloproliferative ziyingozi kodwa emva kwexesha zinokuguquka kwisifo esibi (umdlavuza). Lo mngcipheko wokuba nomhlaza kunye nalezi zifo zibonisa ukubaluleka kokulandelelana okufutshane kunye ne-hematologist yakho.
Ukuhlelwa kwezidalwa ze-myeloproliferative neoplasms kuye kwatshintshile kakhulu kwiminyaka embalwa edlulileyo kodwa siya kuhlaziya iindidi eziqhelekileyo apha.
I-Myeloproliferative Neoplasms
I-"classics" ye-syeloproliferative neoplasms iquka:
- I-Polycythemia vera (PV): Iziphumo ze-PV ezivela ekuguquleni kwezinto eziphilayo ezibangelwa ukwanda kwama-red cells. Maxa wambi i-white cell cell and countlet counts ziphakanyisiwe. Ukuphakama kwezibalo zegazi kwandisa ingozi yakho yokuphuhlisa igazi. Ukuba ufumanisa ukuba unePV, unethuba elincinci lokuphuhlisa i-myelofibrosis okanye umhlaza.
- I-Thrombocythemia ye-Essential (ET): Ukuguqulwa kwemfuza kwi-ET iziphumo kwi-platelet. Inani elongeziweyo leeplatelet ejikelezweni landisa ingozi yakho yokuphuhlisa igazi. Umngcipheko wakho wokuba nomhlaza xa ufumene i-ET incinci kakhulu. I-ET iyingqayizivele phakathi kwe-MPN njengoko ixilongo lokukhutshwa. Oko kuthetha ukuba ugqirha uya kulawula ezinye izizathu zokubala kweplatelet ephakamileyo ( thrombocytosis ) kuquka namanye ama-MPN.
- I-Primary Myelofibrosis (PMF): I- myelofibrosis yaseprayimari iye yabizwa ngokuba yi-idiopathic myelofibrosis okanye i-metoglasia ye-agnoogenic myeloid. Ukuguquka kwemfuyo kwi-PMF kubangela ukutshatyalaliswa (fibrosis) kwintolongo yethambo. Oku kunzima kwenza kube nzima umngeni wakho wethambo ukwenza amangqamuzana egazi amatsha. Ngokwahlukileyo kwi-PV, i-PMF ibangelwa ngokubanzi kwi-anemia (inani elibomvu lentsholongwane yegazi). Inani le-white white cell and countlet count liyakunyuka okanye liyancipha.
- I-Lyeloid Leukemia (CML) engapheliyo : I-CML ingabizwa ngokuba yi-leukemia engapheliyo. Iziphumo ze-CML kwiinguqulelo zofuzo ezibizwa ngokuba yi-BCR / ABL1. Olu tshintsho luphumela ekugqithiseni kwe-granulocytes, uhlobo lweseli elimhlophe legazi. Ekuqaleni, ungenayo impawu kwaye i-CML idla ngokufunyanwa ngokulandelelana kunye nomsebenzi webhabhi oqhelekileyo.
I-Apipical Myeloproliferative Neoplasms
Iimpawu ze-"sophrotheli" ze-syeloproliferative ziquka:
- I-Youth Myleomonocyic Leukemia (JMML): I- JMML yayibizwa ngokuba yi-CML yabantwana. Kuhlobo oluqhelekileyo lwe-leukemia eyenzeka ekusaneni nasebuncinaneni. Umongo weethambo uvelisa iiseli ezimhlophe ezimhlophe ze-myeloid, ngakumbi enye ebizwa ngokuba yi-monocyte (monocytosis). Abantwana abanesifo se-neurofibromatosis I kunye ne-Noonan syndrome basengozini enkulu yokuphuhlisa i-JMML kunabantwana abangenazo izimo zemfuzo.
- I-Leukemia engapheliyo ye-Neutrophilic: I-leukemia engapheliyo ye-neutrophilic isifo esiyintlekele esichazwa ngokunyuka kwama-neutrophils, uhlobo lwama-white white cells. Ezi iiseli ziyakwazi ukungena ngaphakathi kwizitho zakho kwaye zenze ukwandiswa kwesibindi kunye ne-spleen (hepatosplenomegaly).
- I-Eosinophilic Leukemia / i-Hypereosinophilic Syndromes (HES): I-leosemia engapheliyo ye-eosinophilic kunye ne-hypereosinophilc syndromes ibonisa iqela leengxaki ezibonakaliswe ngokunyuka kwenani lama-eosinophil (i-eosinophilia) ebangela ukulimala kwamalungu athile. Inani elithile le-HES lisebenza ngokufana ne-myleoproliferative neoplasms (ke ngoko igama elingapheliyo le-eosinophilic leukemia).
- Isifo sesifo seMast: I-Systemic (ithetha yonke into emzimbeni) isifo sesisitrafini okanye i-mastocytosis yongezwa ngokutsha kwinqanaba le-myeloproliferative neoplasms. Izifo zesininzi zesisiphumo zibangelwa ukwanda kwama-cell cells, uhlobo lweseli legazi elimhlophe liza lihlasele umongo wethambo, umzila wesisu, isikhumba, isilwane kunye nesibindi. Oku kuhambelani ne-mastocytosis enqabileyo echaphazela kuphela ulusu. Amaseli amancinci akhulula i-histamine eyenza ukuba isisombululo sendlela esithintekayo kwiimpawu ezichaphazelekayo.
Imithombo:
URoufosse F, iKlion AD kunye nePreser PF. I-Hypereosinophilic syndromes: Ukubonakaliswa kweklinikhi, ukuvelisa i-pathophysiology kunye nokuxilongwa. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA. (Kufumaneka ngoJuni 27, 2016.)
I-Teferri A. Ingqwalaselo yeeoplasms ze-myeloproliferative. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA. (Kufumaneka ngoJuni 26, 2016.)
Van Eatton RA. Izibonakaliso zekliniki kunye nokuxilongwa kwe-leukemia engapheliyo. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA. (Kufumaneka ngoJuni 28, 2016.)