Anemia, bisa ma'anarsa, ya ƙunshi raguwa a cikin jajayen ƙwayoyin jini, ko matakan haemoglobin. Kalmar “hemolytic anemia” ta ƙunshi nau'ikan anemia daban-daban inda ake lalata jajayen ƙwayoyin jini da wuri a cikin jini. Kalmar "hemolysis" na nufin halakar jajayen ƙwayoyin jini (hemo = jini; lysis = lalata).

Maƙarƙashiyar ƙashi tana da takamaiman iyawar ajiya. Wato yana iya ƙara samar da jajayen ƙwayoyin jini zuwa wani matakin don ramawa da ƙara lalacewa. A al'ada, jajayen ƙwayoyin jini suna yawo a cikin tasoshin jini na kimanin kwanaki 120. A ƙarshen rayuwarsu, saifa da hanta suna lalata su (duba kuma takardar anemia – bayyani). Haɓaka haɓakar ƙwayoyin jajayen jini wani muhimmin abin ƙarfafawa ne a cikin samar da sabbin ƙwayoyin jajayen jini, wanda ke yin sulhu ta hanyar hormone da kodan ke samarwa, erythropoietin (EPO). A wasu lokuta, kasusuwan kasusuwa yana iya samar da jajayen ƙwayoyin jini masu yawa kamar adadin da ya lalace ba bisa ka'ida ba, don haka matakin haemoglobin baya raguwa. Muna magana ne game da diyya hemolysis, ba tare da anemia. Wannan yana da mahimmanci saboda akwai wasu abubuwan da zasu iya haifar da yanayin ya rushe cikin abubuwan da zasu tsoma baki tare da samar da EPO kamar ciki, gazawar koda, rashi folic acid, ko kamuwa da cuta mai tsanani.

Sanadin

An rarraba anemia gaba ɗaya bisa ga ko yana haifar da ita ta hanyar jan jini wanda ita kanta ba ta da kyau (intracorpuscular), ko kuma wani abu wanda yake waje da kwayar jinin jini (extracorpuscular). Hakanan an bambanta tsakanin anemia na gado da samuwar anemia na hemolytic.

Dalilan gado da intracorpuscular

• Haemoglobinopathies (misali sickle cell anemia, da dai sauransu)
• Enzymopathies (misali G6-PD rashi)
• Ciwon ƙwayar mahaifa da cytoskeletal (misali spherocytosis na haihuwa)

Dalilan gado da na waje

• Familial hemolytic-uremic ciwo (na al'ada)

Dalilin da aka samu da kuma intracorpuscular

• Paroxysmal nocturnal hemoglobinuria

Dalili da aka samu da kuma extracorpuscular

• Rushewar injiniya (microangiopathy)
• Wakilai masu guba
• magunguna
• Cututtuka
• Immunological

Bari mu tattauna ƴan misalai to, tun da yake ba shi yiwuwa a kwatanta su duka a cikin mahallin wannan takarda.

Immunological hemolytic anemia:

Halayen autoimmune. A wannan yanayin, jiki, saboda dalilai daban-daban, yana samar da ƙwayoyin rigakafi a kan nasu jajayen ƙwayoyin jini: waɗannan ana kiran su autoantibodies. Akwai nau'i biyu: wadanda ke da autoantibodies masu zafi da wadanda ke da sanyi autoantibodies, dangane da ko mafi kyawun zafin jiki don aikin antibody shine 37 ° C ko 4 ° C. Wannan bambanci yana da mahimmanci saboda magani ya bambanta daga tsari zuwa tsari.

– Zafafan autoantibodies: galibi yana shafar manya kuma yana haifar da anemia na yau da kullun kuma wani lokacin mai tsanani na hemolytic. Suna wakiltar 80% na autoimmune hemolytic anemias. A cikin rabin lokuta, ana iya haifar da su ta wasu kwayoyi (alpha-methyldopa, L-dopa) ko wasu cututtuka (cututtukan ovarian, ciwo na lymphoproliferative, da dai sauransu). Ana kiran wannan a matsayin "na biyu" autoimmune hemolytic anemias, tun da ya bayyana a sakamakon wata cuta.

– Cold auto-antibodies: suna hade da m aukuwa na lalata jajayen kwayoyin halitta da sanyi. A cikin kashi 30 cikin XNUMX na lokuta, muna fama da wani abu na biyu na autoimmune wanda za'a iya bayyana shi ta hanyar kamuwa da cuta ko ƙwayar cuta ta mycoplasma, tsaka-tsakin kwayoyin halitta tsakanin ƙwayoyin cuta da kwayoyin cuta.

Immunoallergic halayen. Idan akwai rashin lafiyar jiki (ba autoimmune) hemolysis na miyagun ƙwayoyi ba, ƙwayoyin rigakafi ba sa kai hari ga ƙwayoyin jajayen jini, amma wasu magunguna: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, da sauransu.

Nau'in hemolytic anemia:

Akwai abubuwa masu mahimmanci guda uku a cikin jajayen ƙwayoyin jini. Akwai haemoglobin, da membrane-cytoskeleton hadaddun, da kuma "injin" enzymatic don sa shi duka aiki. Abubuwan da ba a saba gani ba a cikin kowane ɗayan waɗannan abubuwa uku na iya haifar da anemia na haemolytic.

Abubuwan da ba a saba gani ba na membrane na jajayen ƙwayoyin jini. Babban daya shine spherocytosis na haihuwa, wanda ake kira shi saboda siffar mai siffar zobe wanda sai ya nuna jajayen kwayoyin halitta wanda ke sa su zama masu rauni musamman. Yana da in mun gwada da akai-akai: 1 case a cikin 5000. Da yawa kwayoyin cuta abnormalities suna da hannu, da classic form ne autosomal mamaye, amma recessive siffofin ma wanzu. Yana iya haifar da wasu rikitarwa: gallstones, ulcers a kan kafafu.

Enzymopathies. Akwai nau'i-nau'i da yawa na ƙarancin enzyme wanda zai iya haifar da anemia na hemolytic. Gabaɗaya su na gado ne. Mafi na kowa shi ne rashi a cikin wani enzyme da ake kira "glucose-6-phosphate dehydrogenase", wanda ke haifar da lalacewa da wuri na jajayen jini kuma, daga baya, anemia na hemolytic.

Lalacewar kwayoyin halittar da ke tattare da ita tana da alaƙa da chromosome na X, saboda haka, maza ne kaɗai za su iya shafa. Mata za su iya ɗaukar lahani na kwayoyin halitta kuma su ba da shi ga 'ya'yansu. A cikin mutanen da ke da wannan ƙarancin enzyme, anemia na hemolytic ya fi faruwa bayan bayyanar da abubuwan da ke haifar da iskar oxygen.

Mutanen da ke da rashi G6PD na iya haɓaka hemolysis mai tsanani lokacin da aka fallasa su ga wasu wakilai kamar:

- cin nau'in wake da ake kira ƙananan wake (wake).jaraba faba) ko bayyanar da pollen daga wannan shuka (ana amfani da wannan nau'in wake don ciyar da dabbobi). Wannan hulɗar tana haifar da anemia mai tsanani wanda ake kira favism.

- amfani da wasu kwayoyi: antimalarials, methyldopa (ƙananan jini), sulfonamides (antibacterial), aspirin, nonsteroidal anti-kumburi kwayoyi, quinidine, quinine, da dai sauransu.

– bayyanar da wasu sinadarai irin su mothball.

– wasu cututtuka.

Ana yawan kamuwa da wannan cuta a cikin mutane daga Basin Bahar Rum (musamman tsibirin Girka) da kuma baƙar fata a Afirka da Amurka (inda yawanta ya kai kashi 10% zuwa 14%). A wasu sassan duniya, 20% ko fiye na yawan jama'a suna da shi.

Haemoglobinopathies. Kalmar da aka yi amfani da ita don kwatanta cututtuka na kwayoyin halitta inda aka shafi samar da haemoglobin a cikin kwayoyin jinin jini. Sickle cell anemia (sickle cell anemia) da thalassaemia sune manyan nau'i biyu na haemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Wannan cuta mai tsanani tana da alaƙa da kasancewar haemoglobin mara kyau wanda ake kira hemoglobin S. Wannan yana gurbata jajayen ƙwayoyin jini kuma yana ba su siffar jinjirin jini ko ƙwayar cuta (sickle cell), baya ga haifar da mutuwa. da wuri. Dubi takardar Sickle cell anemia.

Harshen Thalassemia. Ya yaɗu sosai a wasu ƙasashe na duniya, wannan mummunar cuta tana da alaƙa da rashin daidaituwa na kwayoyin halitta wanda ke shafar samar da haemoglobin, wannan launin jini a cikin jajayen ƙwayoyin jini wanda ke ba da damar jigilar iskar oxygen zuwa gabobin. Kwayoyin jajayen jinin da abin ya shafa ba su da ƙarfi kuma da sauri sun karye. Kalmar "thalassemia" ta fito ne daga kalmar Helenanci "thalassa", wanda ke nufin "teku", kamar yadda aka fara gani a cikin mutanen da ke cikin Bahar Rum. Lalacewar kwayoyin halitta na iya shafar wurare biyu a cikin haɗin haemoglobin: sarkar alpha ko sarkar beta. Dangane da nau'in sarkar da abin ya shafa, akwai nau'ikan thalassemia guda biyu: alpha-thalassemia da beta-thalassemia.

Sauran Sanadin

Sanadin injina. Kwayoyin jajayen jini na iya lalacewa yayin wasu jiyya masu alaƙa da na'urorin inji:

- prostheses (bawuloli na wucin gadi don zuciya, da dai sauransu);

- extracorporeal jini tsarkakewa (hemodialysis);

- inji don oxygenate jini (amfani da zuciya-huhu tiyata), da dai sauransu.

Da wuya, mai tseren marathon na iya fuskantar hemolysis na injina yayin da ake murƙushe capillaries a ƙafafu akai-akai. An kuma bayyana wannan halin da ake ciki bayan wasu raye-rayen al'ada da aka tsawaita, a kan ƙafafu.

Fitarwa ga abubuwa masu guba.

- Masana'antu ko samfuran masu guba na gida: aniline, hydrogen arsenic, nitrobenzene, naphthalene, paradichlorobenzene, da sauransu.

– Dabbo mai guba: cizon gizo-gizo, sara, dafin maciji.

– Shuka guba: wasu fungi.

Cutar.Gastroenteritis mai tsanani da ke haifar da shi Kuma coli, cututtukan da ke haifar da pneumococcus ko staphylococcus, hepatitis, zazzabin typhoid, zazzabin cizon sauro, da dai sauransu. Malaria (ko zazzabin cizon sauro) shine mafi mahimmancin sanadin wannan nau'in. Cutar zazzaɓin cizon sauro na faruwa ne ta hanyar ƙwayar cuta da ke girma a cikin jajayen ƙwayoyin jini.

Hyperfunction na saifa. Yana da al'ada don halakar da ƙwayoyin jajayen jini a cikin magudanar bayan tafiyarsu ta kwanaki 120, amma idan wannan sashin jiki yana aiki da yawa, lalata yana da sauri kuma yana haifar da anemia na hemolytic.

Hhemoglobinuria paroxysmal nocturnal. Wannan cuta na yau da kullun yana da alaƙa da kasancewar haemoglobin a cikin fitsari saboda yawan lalata ƙwayoyin jajayen jini. Rikicin dare yana haifar da kowane nau'i na damuwa, ƙarfafa tsarin rigakafi ko wasu magunguna. Wani lokaci cutar tana haifar da ƙananan ciwon baya da rashin jin daɗi.

Matsaloli masu yiwuwa: thrombosis, kasusuwan kasusuwa hypoplasia, cututtuka na biyu.

• Wadanda ke da alaƙa da ƙananan ƙwayoyin jajayen jini: launin fata, gajiya, rauni, juwa, saurin bugun zuciya, da sauransu.
• Jaundice.
• Fitsarin duhu.
• Girman saifa.
• Wadanda suke musamman ga kowane nau'i na anemia na hemolytic. Dubi "Bayyana Likita".

Ga nau'ikan anemia na haemolytic:

• Wadanda suke da tarihin iyali.
• Mutane daga Basin Bahar Rum, Afirka, Kudu da Kudu maso Gabashin Asiya da Yammacin Indiya.

• A cikin mutane tare da rashi na enzyme glucose-6-phosphate dehydrogenase: daukan hotuna zuwa oxidizing jamiái (wasu kwayoyi, filin wake, da dai sauransu).
• Ga sauran nau'ikan anemia na hemolytic:

  – Wasu cututtuka: hepatitis, streptococcal kamuwa da cuta ko E. coli, cututtuka na autoimmune (irin su lupus), ciwon daji na ovarian.

  - Wasu kwayoyi (antimalarials, penicillin, rifampicin, sulfonamides, da dai sauransu) ko kuma masu guba (aniline, arsenic hydrogen, da dai sauransu).

  - Wasu na'urorin inji da ake amfani da su a magani: bawuloli na wucin gadi, na'urori don tsarkakewa ko oxygenating jini.

  - Damuwa.

• A halin yanzu, ba zai yuwu a hana nau'ikan gado ba sai dai ta hanyar tuntuɓar mai ba da shawara kan kwayoyin halitta kafin a haifi ɗa. Kwararrun za su iya tantance haɗarin haifan yaro mai ciwon haemolytic lokacin da ɗaya (ko duka) na iyaye masu yiwuwa suna da tarihin iyali (duba kuma Sickle Cell Anemia don ƙarin cikakkun bayanai game da haɗarin kwayoyin halitta game da wannan nau'i). hemolytic anemia).
• Idan wani abu na musamman yana da alhakin cutar, ya kamata a kauce masa don hana sake dawowa.
• Ga yawancin nau'ikan anemia na hemolytic, yana da mahimmanci a kiyaye wasu cututtuka.

Suna bambanta dangane da nau'in anemia na hemolytic.

• Jiyya ta farko ta dogara ne akan tallafi na gaba ɗaya ga jiki da kuma dalilin da yasa idan zai yiwu
• Ana ba da ƙarin ƙarin folic acid ga marasa lafiya da ke fama da anemia na yau da kullun na hemolytic.
• Alurar riga kafi akan cututtuka na yau da kullun yana da mahimmanci ga marasa lafiya waɗanda ke da lahani na rigakafi, musamman a cikin mutanen da ke da splenectomies (cire sabulu.⁶)
• Wani lokaci ana nuna ƙarin jini
• Wani lokaci ana ba da shawarar splenectomy⁷, musamman a cikin mutanen da ke da spherocytosis na gado, thalassaemias wanda sau da yawa yana buƙatar ƙarin jini amma kuma a wasu lokuta a wasu nau'o'in cutar anemia na kullum. Lallai, galibi a cikin zube ne ake lalata jajayen ƙwayoyin jini.
• Ana wajabta Cortisone wani lokaci don anemia mai zafi na antibody autoimmune kuma don yin la'akari da cutar anemia mai sanyi. Wani lokaci ana amfani dashi a lokuta na haemoglobinuria na nocturnal paroxysmal kuma musamman ga thrombotic thrombocytopenic purpura. Ƙarfafa magungunan rigakafi, irin su rituximab⁸, Immunoglobulins na ciki, azathioprine, cyclophosphamide, da cyclosporine ana iya la'akari da su a cikin anemias hemolytic immunologic. Wani lokaci ana amfani da Plasmapheresis, musamman a cikin yanayin wannan thrombocytopenic purpura.

A matsayin wani ɓangare na ingantacciyar hanyarta, Passeportsanté.net tana gayyatar ku don gano ra'ayin ƙwararren likita. Dr Dominic Larose, likitan gaggawa, yana ba ku ra'ayinsa game da cutar hemolytic anemia :

Magungunan da ba na al'ada ba ne kawai aka gano sun shafi sikila anemia. Dubi wannan takardar don ƙarin bayani.