Myelofibrosis wata cuta ne daga kututture na kasusuwan da zai sa kututture ya zama fibrotic, ko kuma rashin lafiya. Sanda mai tsabta yana cikin ɓarwar jinin jini, a cikin layin tsabta na wasu kasusuwa. Maganin "ƙugi" a cikin myelofibrosis, duk da haka, ba sabanin mawuyacin sakamakon sakamakon warkar da fata ba. Maƙarƙashiya mai cinye shi ne shafin samar da dukkanin jikin jini, don haka tsarin da ya fara dawa ba shi da lahani, kuma zai iya haifar da matsaloli tare da ƙididdigar ƙwayoyin cuta, ƙuntatawa mai tsanani da kuma raguwa na rayuwa.
Lokacin da fibrosis ya karbi, wannan zai haifar da mummunan cutar, kuma yana da mummunar cuta a cikin tantanin halitta kuma wani lokaci a cikin jini. Daga qarshe, a cikin myelofibrosis mai cikewa, wannan zai iya sa kututture ya kasa aiki a gaba ɗaya. Lokacin da myelofibrosis ya cigaba ko ya bunkasa, matsalolin rayuwa kamar kwayar cutar sankarar bargo da jini mai tsanani da kuma zub da jini yana yiwuwa. Haka kuma mawuyacin myelofibrosis zai iya kai ga cutar cutar sankarar bargo , wani ciwon jini na rayuwa mai barazana.
Primary vs. Secondary Myelofibrosis
Myelofibrosis zai iya zama na farko ko na sakandare. Lokacin da ya taso ne, ko kuma an gano shi ne kawai a karo na farko, an kira shi babban myelofibrosis. Lokacin da ya tasowa tare da wasu cututtuka, ciwo ko jinin jini an kira shi myelofibrosis na biyu . Alal misali, myelofibrosis bayan cutar jini da ake kira polycythemia vera ne daya nau'i na na biyu myelofibrosis.
Shin daidai ne a matsayin ciwon daji?
Yana da abubuwa da yawa tare da ciwon daji, amma masana sunyi magana da shi a matsayin "myeloproliferative neoplasm ." Don kiyaye abubuwa mai sauƙi, kalmar neoplasm za a iya dauka a matsayin lokacin zato ga "tsutsa ko ci gaba" da kuma ciwon sukari ko growths zai iya zama m ko m. Myelofibrosis ba lallai ba ne, amma kuma babu wasu daga cikin halaye masu kamala da suka yi tsammanin lokacin da suke tunani game da ciwon daji, ko kuma mummunan ƙwayoyin cuta.
Wannan ya ce, ko kayi la'akari da myelofibrosis a matsayin ciwon daji ko kuma mummunar ci gaban dabbar da ke cikin kasusuwa, yanzu babu magani wanda yake da curative (amma ƙwayar kasusuwa na kasuwa zai iya zama curative), kuma tsari ne wanda zai ci gaba da haifuwa cutar-albeit a gudu daban-daban a cikin mutane daban-daban. Tsarin siffofin myelofibrosis ƙayyade tsawon rai kuma haifar da kima ga lafiyar marasa lafiya.
Za ku sami yalwa da shafukan da suka shafi haƙuri waɗanda ke nuna zuwa ga myelofibrosis a matsayin "ciwon daji na jini." Wannan yana iya zama hanya mai kyau don sadarwa ta al'ada, amma akwai ƙarin labarin. Myelofibrosis zai iya haifar da ciwon jini, amma a wasu lokuta, myelofibrosis zai iya haifar da ciwon jini.
Iri
Bayan na farko da na sakandare, akwai wasu hanyoyin da za a rarraba myelofibrosis. Wata hanya ita ce ta hada da cututtuka na wannan cuta zuwa wasu hadarin haɗari dangane da binciken mutum lokacin da aka gano cutar ta farko. Akwai kayan aiki daban-daban don taimaka wa likitocin ƙayyade ƙimar ku, don taimakawa wajen jagorantar kulawa da kuma tsara siffar ku.
Tsarin jima'i
Bisa ga cutar cutar sankarar bargo da Lymphoma Society , myelofibrosis yana faruwa a kimanin 1.5 daga kowace 100,000 mutane a Amurka a kowace shekara.
Yana shafar maza da mata kuma yawanci ana gano su a cikin mutane fiye da shekaru 60, amma zai iya faruwa a kowane zamani. An kiyasta cewa kimanin mutane 16,000 zuwa 18,500 a Amurka suna da myelofibrosis.
Dalilin
Yawancin nau'in kwayoyin halitta da kwayoyin halitta an san su da hannu, duk da haka, ainihin dalilin da ya fi girma a cikin myelofibrosis ba cikakke ba ne. Yawancin cututtukan kwayoyin halitta da chromosomal sun samo, ciki har da maye gurbin da ake kira "JAK2 V617F maye gurbin," duk da haka wannan maye baya nufin cewa dole ne ka samar da myelofibrosis na farko. Dalilin irin wannan maye gurbin ba shi da sanin, kuma babu wani bayani na musamman ko abubuwan haɗari da za a iya danganta su ga myelofibrosis na farko a mafi yawan lokuta.
Baya ga cututtukan jini, wasu cututtukan jini marasa cututtukan da aka sani da suna "ƙwayoyin cuta na myeloproliferative" kamar su polycythemia vera da kuma muhimmancin thrombocythemia kuma zai iya kai ga na biyu myelofibrosis. Magani na biyu ko myelofibrosis mai mahimmanci zai iya faruwa a sakamakon maganin cututtuka ko rauni na jiki, kamuwa da cuta ko asarar samuwa zuwa kasusuwan kasusuwa.
Myelofibrosis na farko, polycythemia vera-related myelofibrosis, da kuma magungunan myelofibrosis da ake dangantawa da thrombocythemia duk wani lokacin sukan hadu tare da su kamar "myelofibrosis," amma masana kimiyya sun ce akwai yiwuwar koyi da ganewa game da bambancin tsakanin kowane iri.
Cutar cututtuka
Mutane da yawa marasa lafiya basu da wata alamar bayyanar cutar a lokacin ganewar asali, amma alamun sunadaran sun hada da wadannan:
- Wulo
- Asarar nauyi
- Sumi dare
- Fever
- Jin dadin wahalar numfashi
- Rashin jin daɗi a ciki (saboda karar girma)
Ƙarƙashin ɓawon kasusuwa zai iya haifar da bayyanar cututtuka daga ƙananan jini, kamar gajiya daga rashin jinin jini. Hanyoyin da ke tattare da jini suna iya haifar da zub da jini da kuma magance matsaloli.
Sauran cututtuka, irin su cikewar ciki ko matsin lamba, na iya zama saboda duk abin da yake faruwa a waje da kututturer kasusuwa, don yin sabon jini:
- Yawancin lokaci, ƙwayoyin halittar jini suna canzawa zuwa kasusuwa a cikin jarirai a ko kusa da lokacin haihuwa. Kafin haihuwa, duk da haka, jarirai na iya haifar da sababbin kwayoyin jini a shafukan yanar gizo irin su sutura, hanta, da ƙananan lymph-waɗannan shafukan yanar gizo a waje da kasusuwa na kasusuwa ana kiran su ƙaddara.
- Yawanci a cikin tsofaffi, kawai shafin yanar-gizon sabon jini shine kasusuwan kasusuwa. A wasu cututtukan jini da cutar jini, mutane suna komawa wajen samar da jini a duk inda zasu iya, a waɗannan shafukan yanar gizo. A cikin myelofibrosis, wannan ya fi faruwa a cikin ƙwanƙara da hanta. Wani lokuta mutum na iya zama babbar saboda lakaran hematopoiesis a cikin myelofibrosis.
Babban rikitarwa na myelofibrosis kullum yakan fito ne daga kasusuwa daga kasusuwan kasusuwa da kuma hematopoiesis .
Akwai haɗarin hadarin canji ga cutar cutar sankarar myeloid mai zurfi (AML) tare da myelofibrosis, kuma kimanin kashi 20 cikin dari na mutanen da ke dauke da myelofibrosis suna cike da cutar sankarar bargo.
Sanin asali
Bugu da ƙari, bayanin da likitanku ya samu ta hanyar bayyanar cututtuka da jarrabawar jiki, akwai gwaje-gwaje da yawa waɗanda ke bayar da bayanai masu mahimmanci. Wadannan sun haɗa da ƙididdigar jinin jini, wasu aikin jini, gwaje-gwajen hotunan kamar su x-haskoki da MRI, gwaje-gwaje na kasusuwa , da gwajin gwaje-gwaje. Ana iya aika samfurin jini ko ƙashin kasusuwa a dakin gwaje-gwaje don nema maye gurbin (kamar JAK2, CALR, ko MPL maye gurbin) waɗanda suke da yawa a cikin mutane tare da myelofibrosis.
Sauran abubuwa da zasu iya kama da myelofibrosis, amma ba haka ba, sun hada da cutar cutar sankarar jini, da sauran cututtukan myeloproliferative, da cutar cutar sankarar mielomonocytic na kullum, da kuma cutar mukamarin myeloid.
Jiyya
A halin yanzu, babu wani zaɓi na miyagun ƙwayoyi wanda yake da curative. Makasudin mafi yawan marasa lafiya shine don taimakawa bayyanar cututtuka, rage ƙarar girma da kuma inganta yawan ƙwayoyin jini. A cikin haɗin gwiwa tare da waɗannan manufofi, maƙasudin mahimmanci shine kuma ya rage haɗarin rikitarwa.
Myelofibrosis wata cuta ne da ƙananan zaɓuɓɓukan magani, amma ana bincike da ci gaba da yawa. Gudanar da jagorancin maganganun ku na musamman kamar bayyanar cututtuka, da kuma hadarin yanayin ku na musamman na myelofibrosis, har ma a kan shekarunku da kuma cikakken lafiyar ku.
Ga mutanen dake da ƙananan haɗari kuma babu alamun cututtuka, kallo kadai zai iya zama lafiya. Domin rashin lafiyar cutar mai tsanani, ana saran tsinkar dashi daga mai bayarwa , amma duk marasa lafiya ba su dace ba saboda hadarin. Wasu marasa lafiya sune 'yan takara masu kyau don maganin miyagun ƙwayoyi ko magungunan maganin bincike a cikin gwaji.
A shekara ta 2011, Cibiyar Abinci da Drugta (FDA) ta amince da maganin myelofibrosis, ciki har da myelofibrosis na farko, post-polycythemia vera myelofibrosis, da myelofibrosis na thrombocythemia.
- Shawarar FDA ta dogara ne akan sakamakon gwajin gwaji guda biyu da ke cikin marasa lafiya tare da matsakaici ko myelofibrosis kwatanta ruxolitinib zuwa placebo (Nazarin 1) ko kuma mafi kyawun maganin (Nazarin 2). A Nazarin 1, kashi 42 cikin 100 na marasa lafiya da aka bi da ruxolitinib, idan aka kwatanta da kashi 1 cikin dari na marasa lafiya da ake bi da su tare da placebo, sun samu akalla kashi 35 na ragowar girma a cikin makonni 24. A lokacin da aka amince, kashi 75 cikin 100 na marasa lafiya a Nazarin 1 da kashi 67 a Nazarin 2 waɗanda suka samu akalla kashi 35 cikin dari na rage yawan karfin jini sun rage wannan ragewa a cikin ƙarami.
- Ruxolitinib yana da tasiri a rage girman yaduwa da kuma yaduwar cututtuka a yawancin marasa lafiya. A cikin gwajin gwajin gwajin gwaji a cikin marasa lafiya da myelofibrosis mai haɗari, babban ƙungiyar mahalarta na da matukar cigaba a cikin bayyanar cututtuka da ke tattare da cutar: sun rubuta takardun yau da kullum suna daukar nauyin bayyanar cututtuka na myelofibrosis wanda ya hada da rashin tausayi na ciki, jin mamaki na farko, zafi a karkashin hagu na hagu, damuwa, bugu na dare da kashi / tsoka zafi.
- Hanyoyin maganin miyagun ƙwayoyi mafi yawan sunadaran, an kiyasta akalla kashi 1 cikin marasa lafiya da aka bi da ruxolitinib, sun hada da kananan platelets, anemia, bruising, dizziness, da kuma ciwon kai. Hanyoyin maganin miyagun ƙwayoyi na ƙananan ƙoƙari waɗanda suka fi girma a cikin marasa lafiya bi da bi da jinsin da aka kwatanta da placebo a Nazarin 1 sun hada da kananan platelets (sun samu kashi 13 cikin dari na marasa lafiya da aka bi da ruxolitinib, idan aka kwatanta da kashi 1 cikin dari na marasa lafiya da aka saka tare da placebo) da kuma anemia Kashi 45 cikin dari na marasa lafiya da ake bi da su tare da ruxolitinib, idan aka kwatanta da kashi 19 cikin dari na marasa lafiya da ake bi da su tare da placebo). Ana ganin irin wannan sakamakon a Nazarin 2.
Sauran hanyoyin kwantar da hankali sun sami tasiri don kara girma da kuma kula da bayyanar cututtuka sun haɗa da chemotherapies, kaucewa yarinya ko splenectomy, da kuma rashin lafiyar kwayar cutar. Ana iya bayar da jini ga anemia, kuma ga marasa lafiya da suke da transfusion suna dogara da anemia, magungunan kasusuwa na kwayoyi irin su erythropoietin, androgens (alal misali, danazol), da kuma immunomodulators (alal misali, lenalidomide) ana iya amfani dashi.
Faɗakarwa
Bisa ga nazarin da suka gabata, wasu kungiyoyin mutane da aka gano tare da myelofibrosis sun rayu shekaru masu yawa, yayin da a wasu kungiyoyi, lokutan rayuwa sun kasance kasa da shekaru 3 zuwa 5 daga ganewar asali. Kimanin kashi 60 cikin dari na marasa lafiya da na farko / idiopathic myelofibrosis rayu 5 shekaru. Akwai gagarumar rukuni na marasa lafiya, duk da haka, waɗanda suka rayu shekaru 10 ko fiye.
Wadanda suke da fifiko sosai sun haɗa da waɗanda aka samu daga halayen hemoglobin fiye da 10 g / dL, platelet yana ƙidayar fiye da 100x3 / uL da wadanda basu da girma na hanta. Girma da kuma jinsi ba sa alama suna da wata tasiri mai girma akan rayuwa a nazarin, ko da yake a ka'idar, rage girman ƙwayar ƙanƙara zai iya fassarar wadatar amfanin rayuwa a wasu lokuta.
Kalma Daga
Ya zuwa yanzu, rayuwar mutanen da ke dauke da myelofibrosis na farko sun fi dacewa da maganin su da bayyanuwar cutar nan da farko, kuma duk wani magani ko farfadowa; Duk da haka, wannan wani ra'ayi ne a cikin juyin halitta wanda zai canza kamar yadda kimiyya ta bayyana. Sabbin magunguna suna ci gaba da zamawa kuma kimiyya a wannan yanki yana fadada hanzari.
Sources:
> Myelofibrosis: Sabobbin Masarufi ga Ma'aikatan Lafiya: Jagora 2013. Litattafan Scholarly, Jul 22, 2013.
> Gangat N, Caramazza D, Vaidya R, et al. DIPSS-da: A Dynamic International Prognostic Scoring System (DIPSS) don Myelofibrosis na Farko wanda ya hada da Bayanin Tsare-tsaren daga Karyotype, Ƙididdigar Platelet da Tsarin Sanya. J Jara Incol. 2011; 29: 392-397.
> Greenberg PL, Attar E, Bennett JM, et al. Hanyoyin Myelodysplastic: Ka'idojin Nazarin Harkokin Kasuwanci a Oncology. JNCCN. 2013; 11 (7): 838-874.