Waldenstrom ta Macroglobulinemia: cututtuka, Yanã, hangen nesa

Hereditary jini cututtuka ne rare, amma suna matukar hatsari ga mutane. Wata irin wannan cuta ne Vandelstrema macroglobulinemia.

Fiye da shi ne halin?

A karkashin wannan cuta a cikin zamani magani tana nufin wani hereditary hematological ciwo tare da gaban da jini macroglobulin. Wannan gina jiki yana samuwa ta hanyar aiki na monoclonal B-lymphoid jini Kwayoyin (B-lymphocytes).

Yadda aka saba, wadannan Kwayoyin suna da alhakin da kira na jini-M globulin. Lokacin da wani aiki na da bargo (sau da yawa a cikin ci gaban da ƙari tsari) ne wani canji a amino acid abun da ke ciki da kuma jerin gene, abu don kira macroglobulin, Ina nufin tasowa Waldenstrom ta macroglobulinemia.

A cuta ne quite rare, game da 3 lokuta da mutane miliyan. Yana tasowa, yafi a maza wuce shekaru 55 da shekaru. wannan Pathology ne kusan ba ya faruwa har zuwa shekaru 40.

To, ta yaya bayyana cutar a salon salula da kuma nama matakan? Wanne Tsarin shafa da fari da kuma cewa a sakamakon can?

A pathogenesis da cutar

A zuciya na patogenza da cutar ne a m yaduwa na jini Kwayoyin clone Kwayoyin iya tasowa immunoglobulin M. Wadannan Kwayoyin mamaye da bargo, hanta, saifa, inda suka fara zuwa rayayye radiyo da kuma asirce pathogenic macroglobulin. Kamar wancan tasowa Waldenstrom ta macroglobulinemia.

A jari na pathogenic gina jiki a cikin jini take kaiwa zuwa ga cigaban da danko. A sakamakon rage jini ya kwarara kudi ta hanyar tasoshi, wadda take kaiwa zuwa ta stagnation. Bugu da kari, hana jini coagulation dalilai (saboda da Kintsa da kuma inactivate su macroglobulins). Saboda wannan lalace al'ada gudan jini samuwar, abin da kyakkyawan take kaiwa zuwa ƙãra zub da jini.

Gani, idan yayi nazari a karkashin wani madubin, da bargo Kwayoyin, za a iya gani a can suka taru "plazmatizirovannyh" lymphocytes, babban yawan balagagge B-lymphocytes da kuma secreting kumburi shiga tsakani na mast Kwayoyin. Dukkan wadannan Kwayoyin nuna cewa akwai wani Waldenstrom macroglobulinemia.

cututtuka

Ta yaya za a iya asibiti a da ake zargi da wannan cuta?

A farko marasa lafiya za su yi kuka da nonspecific cututtuka - janar wani rauni, low-sa zazzabi na dogon lokaci, sweating, nauyi asara wani kwakkwaran dalili ba.

Babban alama, a kan tushen da wanda za mu iya zargin cewa samo asali Vandelstrema macroglobulinemia, ta ƙara zub da jini daga hanci, gumis da kuma mucous membranes. Da yawa kasa akai-akai lura subcutaneous bruises da kuma bruises.

A tsawon lokaci, iya ci gaba hepatosplenomegaly da lymphadenopathy (kumbura Lymph nodes). Shi ne kuma zai yiwu a ci gaba da rikitarwa macroglobulinemia.

Lokacin da gazawar samar dace kula da lafiya, akwai wani hadarin wani coma haƙuri, har ma da mutuwa (amma mutuwa marasa lafiya ne yawanci ba sosai a kan abin da suke fama da Waldenstrom ta macroglobulinemia). A Sanadin ga mutuwar ne sakamakon ci gaba da rikitarwa.

rikitarwa da cutar

A wannan za a iya wuya da cuta?

Da farko da hannu a cikin pathological aiwatar da zuciya da jijiyoyin jini tsarin, wato da kananan tasoshin - capillaries, arterioles da venules. By rage jini ya kwarara lura da ci gaban retinopathy, nephropathy. Koda lalacewa. A wannan yanayin halin da ci gaban glomerular jijiyoyin bugun gini occlusion da urolithiasis da shaida na urate.

Macroglobulinemia Waldenstrom halin low rigakafi da kuma shiga opportunistic cututtuka complicating da bincikowa ga tamkar cuta.

Kamar yadda ci gaban da aiwatar da shi ne alama danniya na kusan duk germs na hematopoiesis da kuma ci gaban pancytopenia. Amyloidosis halin da ci gaba da kuma pathological raunuka cewar gina jiki hanta da saifa jini, wanda kara worsens da haka su illa aiki.

bincikowa da

Abin da awo zai taimake ka ka fahimci cewa samo asali macroglobulinemia?

Da farko, ya kamata mu kula da CBC. Babban Manuniya da cutar za su kara a erythrocyte sedimentation kudi da kuma samuwar takamaiman "rouleaux" - mai danko tare da ja da maikacin jini. Wbc yawanci nuna wani ƙara yawan lymphocytes a cikin bincike da kuma bayyanar m leukocytes siffofin.

Don tabbatar da ganewar asali, wajen gudanar da immuno. Bayan gudanar da wannan binciken ba zai iya gane wani ƙara adadin a cikin jini immunoglobulin M

Monoclonal immunoglobulins aka ƙaddara a cikin jini bayan Bugu da kari na labeled Sera.

Ƙarin, amma ba na musamman cututtuka sun hada da wani kara girman hanta da saifa, ganewa na amyloid a biopsy kappillyarah koda, kazalika da rage yawan jini coagulation dalilai (musamman, factor 8).

magani

A farkon matakai da cutar, sai dai in akwai gagarumin ciki sashin jiki hannu, musamman magani ba a nuna. Sau da yawa, wadannan marasa lafiya ne a karkashin likita dubawa a gundumar hematologist.

Lokacin na farko da bayyanar cututtuka da kuma tabbatar da cewa cutar - Waldenstrom macroglobulinemia, za a fara takamaiman far da cytostatics. Ga magani amfani da kwayoyi kamar "hlorbutin", "Cyclophosphamide". Bã su da wani cytostatic sakamako da kuma kashe da ayyukan da wani m clone na B-lifotsitov.

Nada a farkon wuri "hlorbutin" to 6 MG kullum da baki ga 3-4 makonni. Kara kashi ne fraught tare da ci gaban da bargo aplasia. Bayan kammala da hanya na lura wajabta tabbatarwa allurai da miyagun ƙwayoyi (4.2 MG) da duk sauran rana.

Don inganta jini rheological sigogi amfani plasmapheresis. plasmapheresis magani ne da za'ayi domin yin amfani da cytotoxic kwayoyi. Ga kau hanya da aka nuna a cikin 2 lita jini tare da bayarwa canzawa a kan ta inactivated.

rigakafin

Saboda cutar ne hereditary, yana da wuya a kowace hanya shafi ta ci gaba, tun da shi an riga an shar'anta a cikin kwayoyin halittu. A Iyakar hanyar gujewa ci gaban dace ganewar asali na cutar da kuma da-kõma shirin magani.

Kaikaice shafi cutar zai iya zama batun da lafiya salon, bada up shan taba da kuma shan giya, iyaka kyafaffen da yaji jita-jita.

Tun da cuta shi ne kayyade, shi zai iya fararwa ta ci gaba da kuma wasu mummunar yanayi - aiki tare da aniline dyes, Paints, varnishes.

M kwayar cututtuka ma iya canza mutum genome. Farkon lura da wadannan cututtuka zai taimaka wajen hana canje-canje a cikin kwayoyin halittu da kuma hana ci gaban macroglobulinemia.

M matakan za a iya dangana a familiarize marasa lafiya tare da siffofin da cutar da kuma gabatarwa da ayyukan su cika da lafiya salon.

hangen nesa

Abin jira don marasa lafiya da suke sun ɓullo da Waldenstrom macroglobulinemia? Hangen nesa da cutar dogara da tsanani da cuta da cewa sun ci gaba da rikitarwa da kuma dace magani.

Idan cutar ne a cikin farkon mataki na ci gaba, a mafi yawan lokuta yana yiwuwa su hana ta ci gaba. Irin wannan marasa lafiya ko da suna da wata dama ga cikakken magani, da yin amfani da tabbatarwa allurai cytostatics har yanzu za a iya muhimmanci ƙara da rayuwar jama'a.

Shi ne mafi sharri ga waɗanda suke tãre da rikitarwa Waldenstrom ta macroglobulinemia. Bayyanar cututtuka, da hangen nesa da wanda yake shi ne musamman m - amyloid hanta da koda, zub da jini da kuma m cin paraproteinemic coma. Idan lokaci ba ya taimaka irin marasa lafiya, wani babban yiwuwar mutuwa.

A tsawon rayuwar jama'a ga marasa lafiya da cutar ne game da 4-5 shekaru. Lokacin da yadda ya kamata jawo sama da shirin na lura iya kara shi don 9-12 shekaru.

Hadarin cuta

Ganin cewa cutar na faruwa da wuya, yana da wuya a zargin shi na ci gaban wani mutum wanda kusan ba ya sadu da shi. Ganin cewa na farko lamba tare da haƙuri ta gida therapists, shi ne a kafaɗunsu, kuma shi ne ke da alhakin tabbatar da cewa dace zargin da bincikar lafiya tare da Waldenstrom ta macroglobulinemia. Menene wannan cuta, za a tuna, ba dukkan likitoci, duk da haka, ganin cewa haƙuri da ya shigo ba Pathology, shan la'akari duk da na asibiti manifestations, ya kamata duk wani ilimin.

A nan gaba, wannan haƙuri zai kai hematologists, amma farko ganewar asali ya ginu ne kawai a kan kafadu da likitoci kananan dakunan shan magani.

Late ganewa da cutar take kaiwa zuwa tsanani sakamakon lokacin jiyyar cutar sankara ne ba tasiri. Wannan shi ne dalilin da ya sa ya kamata ka sani game da wannan cuta duk ba miss shi, kuma kada ku kawo a jihar na sakaci.