Waldenstrom a Macroglobulinemia: zizindikiro osiyanasiyana, madokotala ananena zawo

matenda otengera magazi n'zochepa, koma ndi oopsa kwambiri kwa anthu. matenda china Vandelstrema macroglobulinemia.

Kuposa amakhala?

Mu matenda mankhwala amakono amanena za cholowa hematological syndrome limodzi ndi kukhalapo kwa macroglobulin magazi. mapuloteni Izi aumbike mwa ntchito za monoclonal maselo magazi B-lymphoid (B-lymphocytes).

Mwachibadwa, maselo amenewa ali ndi udindo kaphatikizidwe wa magazi-M globulin. Pamene ntchito iliyonse ndi mafupa (zambiri mu chitukuko cha ndondomeko chotupa) ndi kusintha kwa amino asidi kapangidwe ndondomeko ya jini ya, zikubweretsa kaphatikizidwe macroglobulin, i.e. akutukuka macroglobulinemia Waldenstrom a.

Matendawa ali osowa kwambiri, za 3 milandu pa anthu miliyoni. Akufotokozera makamaka amuna ndi zaka 55. kudwala izi pafupifupi zimachokeradi zaka 40.

Nanga kuwonetsera matenda pa milingo ma ndi minofu? Amene nyumba amakhudzidwa mu malo oyamba ndi kuti chifukwa cha kumeneko?

The pathogenesis matendawa

Pa mtima wa patogenza matenda ndi kuchuluka zilonda maselo plasma choyerekeza maselo amatha kukulitsa immunoglobulin M. Maselo amenewa angalowe mafupa, chiwindi, ndulu, pamene iwo anayamba mwachangu zikupitiriza ndi secrete tizilombo macroglobulin. Izi zinachititsa macroglobulinemia Waldenstrom a.

Kudzikundikira mapuloteni tizilombo m'magazi kumabweretsa chitukuko cha kukhuthala ake. Chifukwa cha kuchepa kwa ndalama otaya magazi kudzera ziwiya, imbaenda stagnation ake. Komanso, imathetsa plasma coagulation zinthu (chifukwa okutira ndi inactivate iwo macroglobulins). Chifukwa cha ichi akusokoneza yawamba aumirira mapangidwe, zomwe mapeto chinawonjezeka magazi.

Zowoneka, bwinobwino simungathe kutiona, maselo mafupa, Tingaone kumeneko accumulations "plazmatizirovannyh" lymphocytes, ambiri okhwima B-lymphocytes ndi secreting owayimira yotupa maselo mlongoti. Onse a maselo amenewa akusonyeza kuti pali Waldenstrom macroglobulinemia.

zizindikiro

Kodi kukomoka kuti amaganiziridwa matenda?

Odwala choyamba amadandaula zizindikiro nonspecific - ambiri kufooka, malungo otsika kalasi kwa nthawi yaitali, thukuta, kuwonda popanda chifukwa chenicheni.

The chizindikiro chachikulu, pa maziko amene ife tikhoza kuganiza kuti kusanduka Vandelstrema macroglobulinemia, ndi chinawonjezeka magazi kuchokera mphuno, m'kamwa ndi nembanemba mucous. Kwambiri kamodzikamodzi anati mikwingwirima subcutaneous ndi mikwingwirima.

M'kupita kwa nthawi, angathe hepatosplenomegaly ndi lymphadenopathy (kutupa lymph mfundo). N'zothekanso chitukuko cha mavuto macroglobulinemia.

Pamene kulephera kupereka chithandizo yake pali chiopsezo cha chikomokere mtima, ngakhalenso imfa (koma akufa odwala amakhala osati kwambiri zimene iwo akudwala macroglobulinemia Waldenstrom a). Zimayambitsa imfa yawo ndi chifukwa cha mavuto anayamba.

mavuto a matenda

N'chimodzimodzinso ndi kobvuta ndi matenda?

Makamaka amene amasamalira pathological a dongosolo mtima, mosonyeza ziwiya kakang'ono - capillaries, arterioles ndi venules. Ndi kuchepetsa magazi anati chitukuko cha retinopathy, nephropathy. Impso kuwonongeka. Pankhaniyi yodziwika ndi chitukuko cha glomerular mtima occlusion ndi urolithiasis ndi mafunsidwe a urate.

Macroglobulinemia Waldenstrom yodziwika ndi chitetezo chokwanira otsika ndi kujowina matenda osadziwika complicating ndi diagnostics matenda chachikulu.

Monga kupitirira kwa ndondomekoyi ndi Amowabu yimitsira wa pafupifupi majeremusi onse hematopoiesis ndi chitukuko cha pancytopenia. Amyloidosis yodziwika ndi chitukuko ndi zotupa pathological Malinga mapuloteni chiwindi ndi ziwiya ndulu magazi, zomwe zina worsens ndi ntchito zawo mkhutu.

diagnostics

Kodi zokhazikitsidwa adzakuthandizani kumvetsa chomwe chinasintha macroglobulinemia?

Choyamba, tiyenera kulabadira kwa malamulowa. Zowonetsera matenda adzawonjezera mlingo sedimentation erythrocyte ndi mapangidwe yeniyeni "rouleaux" - kusagwirizana maselo a magazi. Wbc ambiri limasonyeza kochulukira chiwerengero cha lymphocytes mu kusanthula ndi maonekedwe a mwana leukocytes mitundu.

Kutsimikiza matenda mwa kuchititsa immuno. Pambuyo phunziro ili simungathe kudziwa kuchuluka zochuluka immunoglobulin magazi M

immunoglobulins Monoclonal atsimikiziridwa magazi pambuyo Kuwonjezera zaikidwa Sera.

Zina, koma osati yeniyeni zizindikiro monga ndi chiwindi zikuluzikulu ndi ndulu, kudziwika amyloid mu biopsy kappillyarah impso, komanso kuchepetsa chiwerengero cha zinthu plasma coagulation (makamaka, chinthu 8).

chithandizo

Mu magawo oyambirira a matenda, ngati pali kwambiri mkati limba okhudzidwa, mankhwala enieni si anasonyeza. Nthawi zambiri, odwala awa moyang'aniridwa ndi achipatala pa chigawo panthawiyi, dokotala.

Pamene zizindikiro woyamba n'kutsimikizira kuti matenda - Waldenstrom macroglobulinemia, anayamba mankhwala enieni ndi cytostatics. Kwa mankhwala mankhwala ntchito monga "hlorbutin", "Cyclophosphamide". Iwo ndi zotsatira cytostatic ndi kupondereza ntchito ya choyerekeza zilonda za B-lifotsitov.

Akusankhidwa malo oyamba "hlorbutin" 6 mg ndi tsiku pakamwa kwa masabata 3-4. Akawawonjezere mlingo ndi zinadzala ndi chitukuko cha mafupa aplasia. Nditamaliza njira ya mankhwala zotchulidwa Mlingo yokonza mankhwala (4.2 mg) tsiku lililonse.

Kuonjezera magazi magawo rheological ntchito plasmapheresis. plasmapheresis mankhwala ikuchitika ntchito mankhwala cytotoxic. Kuchotsa ndondomeko tikuonera 2 malita madzi ndi mwiniwake M'MALO pa inactivated ake.

kupewa

Chifukwa matenda ndi cholowa, n'zovuta mwa njira iliyonse bwanji chitukuko, popeza kale anaika mu chibadwa. Njira yokhayo kupewa chitukuko cha matenda yake ya matenda ndi bwino okokedwa dongosolo la mankhwala.

Mwanjira bwanji matenda gonjerani moyo wathanzi, kusiya kusuta fodya ndi kumwa mowa, kusuta malire ndi mbale zokometsera.

Popeza matenda ndi chibadwa, izo lingachititse chitukuko ndi zina zinthu zoipa - ntchito ndi utoto aniline, utoto, vanishi.

Matenda kawirikawiri tizilombo amathanso kusintha matupi a anthu. mankhwala oyambirira a matenda awa kupewa kusintha majini ndi kulepheretsa chitukuko cha macroglobulinemia.

zodzitetezera akhoza zimachitika dziŵani odwala ndi mbali ya matenda ndi kulimbikitsa ntchito zawo mogwirizana ndi moyo wathanzi.

madokotala ananena zawo

Kodi amadikirira odwala amene apanga Waldenstrom macroglobulinemia? Kulosera tsogolo la matenda a matenda zimadalira kukula kwa matendawa kuti apanga mavuto ndi chithandizo yake.

Ngati matenda ali titangoyamba chitukuko, nthawi zambiri n'zotheka kuteteza patsogolo ake. odwala umenewu ndi mwayi mankhwala wathunthu, ntchito Mlingo yokonza cytostatics ukhoza kwambiri kuonjezera moyo wawo amayembekezeka.

Ndi zikuipiraipirabe kuti anthu macroglobulinemia zovuta Waldenstrom a. Zizindikiro, kuti madokotala ananena zawo zomwe ndi zokhumudwitsa kwambiri - amyloid chiwindi ndi impso, magazi pafupipafupi chitukuko paraproteinemic chikomokere. Ngati nthawi si kuthandiza odwala amenewa, mwina mkulu wa imfa.

Wapakati moyo amayembekezeka kwa odwala matenda ndi za 4-5 zaka. Pamene bwino kukafola dongosolo la mankhwala achuluke kuti zaka 9-12.

Ngozi ya matenda

Popeza kuti matenda kumachitika kawirikawiri, n'zovuta kuganiza iye chitukuko cha munthu amene pafupifupi konse adakomana naye. Popeza kuti yoyamba ndi wodwala madokotala m'deralo, ndi pa mapewa awo, ndi udindo kuonetsetsa kuti yake anali amaganiziridwa ndipo anapezeka ndi macroglobulinemia Waldenstrom a. Kodi matenda, sizidzakumbukiridwa, osati onse madokotala Komabe, ndikuganiza kuti wodwalayo ali nditangokamba kudwala, kutenga nkhani mawonetseredwe zake zonse matenda, kodi dokotala aliyense.

M'tsogolo, mtima adzatsogolera hematologists, koma matenda chachikulu apuma chokhazikika pa mapewa a zipatala madokotala.

Mochedwa chizindikiritso cha matenda kumayambitsa mavuto aakulu pamene mankhwala amphamvu salinso ogwira. N'chifukwa chake muyenera kudziwa za matenda onse kuti muphonye izo ndipo sizibweretsa ku mkhalidwe osasamalidwa.