Agranulocytosis - dab tsi yog qhov no? Agranulocytosis nyob rau hauv cov me nyuam thiab cov neeg laus: Cov tsos mob, ua rau thiab cov kev kho

Kuaj kev soj ntsuam ntawm cov ntshav ua zoo txhua txhua tus kab mob. Qhov no yog kev cob cog rua nrog cov ntaub ntawv ntsiab lus ntawm cov tshuaj ntsuam. Nyob rau kev hloov ntawm ib tug neeg tsis muaj ntshav yuav pom nyob rau lub etiology ntawm tus kab mob, heev ntawm lub pathological txheej txheem. Cov ntaub ntawv nyob rau hauv qhov kev nce los yog txo nyob rau hauv tej yam kev ntshav hlwb tso cai kev xav tias kev ua txhaum. Piv txwv li, leukemia, ntau hom ntawm anemia. Ib kuaj tej yam tshwm sim xam tau tias yog agranulocytosis. Qhov no syndrome, uas tej zaum yuav tshwm sim nyob ib leeg los yog nyob rau hauv ua ke nrog nrog rau lwm cov kev hloov nyob rau hauv cov ntshav. Qhov no kos npe rau hais tias tus muaj mob kab mob.

Agranulocytosis - dab tsi yog qhov no?

Kab mob ntawm cov ntshav muab kev koom tes nyob rau hauv science hematology. Kev cuam tshuam, uas qhia mob loj heev pathological txheej txheem yog agranulocytosis. Qhov no syndrome yog yus muaj los ntawm ib tug qhia tau txo nyob rau hauv cov theem ntawm cov ntshav dawb - leukocytes. Lawv yog cov tsim nyog rau cov kev tswj ntawm kab agents nkag mus rau hauv lub cev thaum lub sij hawm kab mob.

Qhov ntshav dawb count yog sib npaug zos rau 4-9 x 10 ⁹ / l. Kom nyob rau hauv tus naj npawb ntawm cov hlwb qhia txoj kev loj hlob ntawm lub inflammatory kom pom tseeb. Qhov uas tsis muaj ntshav dawb yuav tshwm sim thaum lub tsis ua hauj lwm ntawm lub cev, inhibition ntawm hematopoiesis, intoxication. Agranulocytosis - ib tug kuaj syndrome yus poob nyob rau hauv lub theem ntawm cov ntshav dawb los 1 x 10 ⁹ / l los yog tsawg dua (Neutrophil - 0.75 * 10 ^9). Vim lub pronounced deficiency ntawm lub cev hlwb muaj ntau ntau yam dab kis kab mob los yog kab etiology. Yuav kom qhia tau tias cov syndrome, soj ntsuam ntshav kuaj ntawd yog tsim nyog.

Tus zaus ntawm tshwm sim ntawm agranulocytosis

Qhov no hematologic syndrome tshwm sim ntawm cov poj niam thiab txiv neej pejxeem. Congenital agranulocytosis tsis tshua kaw, nyob rau hauv kev sib piv nrog rau cov kis tau tus kab mob. Ib yam li ntawd, kev hloov nyob rau hauv lub cellular muaj pes tsawg leeg ntawm cov ntshav nws muaj ntsis mus rau theem nrab-hnub nyoog poj niam (los ntawm 40 xyoo). Tus zaus ntawm no lab feature yog txawv nyob rau hauv ntau lub teb chaws. Nyob rau nruab nrab, agranulocytosis kuaj nyob rau hauv 1 ntawm 1200 tus neeg. Ib tug tseem ceeb yuav txo tau nyob rau hauv lub xov tooj ntawm cov ntshav dawb ntau nws Caucasians.

Vim li cas rau txoj kev loj hlob ntawm agranulocytosis

Yog vim li cas tsim agranulocytosis? Yog vim li cas rau qhov no pathological mob nyob rau hauv lub ntau yam ntawm tus kab mob. Congenital agranulocytosis tshwm sim los ntawm roj ntsha yam. Lub syndrome hais txog kom txhob kaw txiav txim pathologies. Cov kev pheej hmoo tias tus me nyuam yug los ntawm ib tug neeg muaj mob leej niam los yog leej txiv, yuav muab qhov chaw no syndrome no heev. Yog vim li cas yuav ua tau rau kis tau agranulocytosis muaj xws li:

1. Cov kab mob autoimmune. Tej pathologies yog mauj lupus erythematosus, scleroderma, mob caj dab rheumatoid. Nyob rau hauv cov kab mob, lub cev tiv thaiv yog tsis tau tsuas yog aimed ntawm txawv teb chaws li hais, tab sis kuj nyob rau hauv lub cev tus kheej hlwb.
2. Raug ionizing tawg. Qhov no zoo tshaj ua rau yus tsim txom tsis yog tsuas yog dawb, tab sis tseem liab hematopoietic kab.
3. Tau txais cytostatic tshuaj. Cov medicaments yog qhia rau rheumatic kab mob thiab mob cancer. Lub disadvantage ntawm kev kho mob nrog cytostatics yog tias cov tshuaj puas ib txwm hlwb.
4. Long-term siv tshuaj tua kab mob (e.g., tuberculosis), narcotics yuav muab thiab antiparasitic agents.
5. Loj kab mob. Tus ntse poob nyob rau hauv lub xov tooj qe yuav tsum tau cai nyob rau hauv kab kab mob siab, qhua pias, rubella, smallpox, mob khaub thuas, thiab hais txog. D.

Nws yog ib nqi sau cia hais tias lub agranulocytosis tsis yog ib qho kev ywj siab tus kab mob. Qhov no syndrome yuav tsis tsim tsis muaj ib tug kev vim li cas. Nws yog ib txwm txuam nrog ib co pathological los.

Lub mechanism ntawm kev loj hlob ntawm agranulocytosis

Neutrophils yog tus tiv thaiv cov ntshav. Lawv tsim nyob rau hauv lub hlwb pob txha thiab tso tawm nyob rau hauv loj qhov ntau tsis tu ncua. Cov hlwb dab tsi rau txog 70% ntawm tag nrho cov leukocytes. Nrog cov tsis muaj txoj kev loj hlob ntawm cov ntshav ntawm cov ntsiab yog tsim neutropenia. Agranulocytosis xam tau tias yog advanced ntaub ntawv ntawm qhov mob. Congenital tsis muaj peev xwm ntawm cov Neutrophil thiab tag nrho cov dawb cell count tshwm sim vim muaj cov qhov txawv txav noob.

Raws li ib tug tshwm sim ntawm exogenic yam, txoj kev loj hlob ntawm cov ntshav dawb yog txo los yog tso ntawv tseg tsis kiag li. Lwm mechanism ntawm lub syndrome yog suav tias yog zus tau tej cov tshuaj mus rau lub qe. Yog li, muaj agranulocytosis. Kuaj ntshav nyob rau tib lub sij hawm yuav hloov yuav luag tam sim ntawd. Tom qab tag nrho, thaum lub sij hawm lub neej ntawm qe es luv luv. Nws yog los ntawm 2 mus rau 3 hnub.

Ib suam ntawm pathological tej yam kev mob

Nyob ntawm seb qhov ua rau thiab pathogenetic mechanism paim ob peb classifications agranulocytosis. Raws li rau qhov no, raug rho tawm congenital thiab nrhiav tsis muaj peev xwm qe. Nyob rau hauv thawj cov ntaub ntawv, cov Hematological syndrome tshwm sim thaum lub nram qab no caj pathologies:

1. Granulomatous kab mob.
2. Familial neutropenia.
3. Shvahmana-Dayemonda syndrome.
4. Kostmann kab mob.
5. Syndrome Shediac-Shtaynbrinka-Higashi.
6. Congenital aleukia.

Nrhiav tsis muaj peev xwm ntawm cov ntshav dawb yog muab faib mus rau hauv autoimmune, thiab hapten myelotoxic agranulocytosis. Txhua yam ntawm cov ntaub ntawv nws muaj nws tus kheej mechanism ntawm txoj kev loj hlob. Downstream pathological txheej txheem yog cais mob thiab ntev lub xeev agranulocytosis. Tsis tas li ntawd nyob rau hauv lub cais yog raws li nyob rau hauv lub heev ntawm lub syndrome.

Hauj lwm tiv thaiv kab mob agranulocytosis

Immune agranulocytosis txuam nrog rau pathological los ntawm cov tshuaj nyob rau hauv cov ntshav liab. Nws muaj peev xwm muab tau los ntawm ob leeg endogenous thiab exogenous mechanism. Nyob rau hauv thawj cov ntaub ntawv, tus kab mob no tshwm sim vim autoimmune nres. Xws li ib tug lub xeev ua rau mauj connective cov ntaub so ntswg mob. Immune system hlwb tsis hloov nyob rau hauv muaj pes tsawg leeg, tab sis lawv yog raws li nws twb "reprogrammed." Raws li ib tug tshwm sim, lawv pib tawm tsam kab mob tus kheej ntaub so ntswg. Granulocytes sai sai mature thiab nws tus kheej-kev puas tsuaj. Exogenous yam nyob rau hauv cov ntaub ntawv no yog sob. tiv thaiv kab mob agranulocytosis syndrome tshwm sim nyob rau hauv xws pathologies li mauj lupus erythematosus thiab rheumatoid mob caj dab. Nyob rau tib lub sij hawm tshaj qe nrog rau ib tug tsis muaj lwm yam ntshav.

Lwm variant ntawm tus kab mob, uas tshwm sim nyob rau hauv lub keeb kwm yav dhau ntawm lub cev muaj dab, ib tug hapten agranulocytosis. Nyob rau hauv daim ntawv no ntawm tus pathological txheej txheem kuj ua ib tug antibody rau qe. Txawv yog hais tias uas lawv ua yog nyob ib leeg, thiab tom qab noj tej yam tshuaj - hapten. Ua kom tsawg ntawm cov tshuaj ua ib lub cev tsis teb, hais nyob rau hauv granulocytes kev puas tsuaj. Lub haptens tau ua raws li ib co tshuaj tua kab mob, antiinflammatory thiab hypoglycemic agents, barbiturates.

Hauj lwm myelotoxic agranulocytosis

Myelotoxic agranulocytosis npaj raws li ib tug tshwm sim ntawm exogenous influences. Nws yeej ib txwm ua ke nrog erythromycin thiab thrombocytopenia. Nyob rau hauv feem ntau tus neeg mob, myelotoxic variant ntawm tus kab mob yog txuam nrog raug chemotherapeutic neeg thiab ionizing tawg. Tej yam zoo coj mus rau inhibition ntawm hematopoiesis nyob rau hauv lub qis dua theem ntawm nws txoj kev loj hlob. Mus rau cytostatic tshuaj uas ua rau zoo xws li cov tshuaj tiv thaiv hais txog cyclophosphamide, fluorouracil, mercaptopurine, thiab lwm yam D. inhibition ntawm hematopoietic progenitor hlwb ua rau ib tug txo nyob rau hauv lub xov tooj ntawm granulocytes nyob rau hauv lub hlwb pob txha. Qhov no yog vim lub maturation ntawm cov ntshav mob cov kab mob.

Nyob rau hauv tas li ntawd mus exogenous influences, tsis muaj hematopoietic hlwb muaj nyob rau hauv ib co hom anemia thiab leukemia. Cov yog vim li cas feem ntau tshwm sim los agranulocytosis nyob rau hauv cov me nyuam. Feem ntau, hematologic hloov yog raws roj ntsha. Piv txwv xam tau tias yog hemolytic thiab aplastic anemia, Fanconi lub syndrome. Mob thiab ntev myelogenous leukemia muaj nyob rau hauv thaum yau thiab nyob rau hauv cov neeg laus cov pejxeem.

Agranulocytosis: cov tsos mob

Tsis txaus granulocytes nrog mob loj heev kev soj ntsuam ces. Tej zaum lawv yuav sib txawv, nyob ntawm seb lwm tus kab mob, uas coj mus rau tshwm sim ntawm Neutrophil tsis muaj peev xwm. Yuav ua li cas puas agranulocytosis? Cov tsos mob ntawm tus kab mob:

1. Loj tsis muaj zog.
2. High kub ib ce.
3. Lymphadenopathy.
4. Hepatosplenomegaly.
5. Necrotic txhab ntawm lub qog ua kua week.
6. Hemorrhagic syndrome.

Hapten agranulocytosis, tsis zoo li lwm cov ntaub ntawv, yog yus muaj los ntawm ib tus mob kawg. Cov neeg mob mob nyhav ntxiv sai sai npaj ib kub taub hau, muaj qhov ncauj tawm nyob rau hauv lub qhov ncauj. Puas pos hniav, tonsils, uvula, mos palate loj zuj zus, ua rau cov ntaub so ntswg necrosis. Nws yog nrog los ntawm kev mob, tsis muaj peev xwm ntawm nqos, ntev li salivation.

Thaum myelotoxic agranulocytosis variant feem ntau tsim los ntshav. Lawv muaj kev cob cog tsis tau tsuas yog nrog ulceration ntawm lub qog ua kua week, tab sis kuj nrog platelet tsis muaj peev xwm. Ntaus cim qhov ntswg, gingival, uterine, hnyuv los ntshav. Nyob rau hauv daim tawv nqaij yuav tshwm sim hematoma. Vim lub rwj-necrotic dab nyob rau hauv cov hnyuv cai raws plab, mob plab. Raws li ib tug tshwm sim ntawm aspiration ntawm cov ntshav muaj hemorrhagic mob ntsws dej.

Cov kev hloov nyob rau hauv lub seev nyob rau ntawm ib agranulocytosis

Feem ntau agranulocytosis txoj kev loj hlob tshwm sim nyob rau hauv cov neeg mob uas mob cancer, mauj thiab loj kis kab mob. Qhov no syndrome yuav xav tias tej yam ntxwv raws li kub taub hau, qaug zog thiab cov tsos ntawm ulcers nyob rau hauv lub qhov ncauj. Nws yog yooj yim dua rau kev tshawb nrhiav congenital agranulocytosis. Ib tug sim ntshav xyuas tsim nyog ua nyob rau hauv kev mob loj pallor ntawm daim tawv nqaij ntawm tus me nyuam, hemorrhagic ces. Nyob rau hauv qhov kev tshawb nrhiav yog ib tug txo ntawm leukocytes (tsawg tshaj li 1 × 10 ⁹ / l). nws yog feem ntau txuam nrog anemia thiab thrombocytopenia.

Yuav kom nrhiav tau tawm, txawm yog vim li cas, tsim lub Hematological syndrome, ua immunogram tej thiab hlwb pob txha. Yuav kom tshawb nrhiav qhov teeb meem tshwm sim los ntawm ib tug tsis muaj peev xwm ntawm granulocytes nqa tawm instrumental kev tshawb fawb. Cov lawv - lub hauv siab X-ray, xoos hnyuv laus. Nws kuj yuav tsum tau tus kws pab tswv yim - otolaryngologist, oncologist, kws kho hniav, rheumatologist thiab gastroenterologist.

Pathology Txoj kev ntawm kev kho mob

Yuav tsum tau tus neeg mob pw tsev kho mob, yog pom agranulocytosis. Kev kho mob muaj nyob rau hauv kws kho ulcerous thiab necrotic chaw antiseptic agents, tshuaj pleev npaj. Rau tus neeg mob yog tsis raug mus rau ib tug thib ob tus kab mob, nws yog muab tso nyob rau hauv lub chamber ntawm lub isolator. Yog hais tias puas plab hnyuv mucosa yog powered los ntawm cov parenteral lawv.

Cov tshuaj rau cov kev kho mob ntawm agranulocytosis

Yuav kom tiv thaiv cov kev loj hlob ntawm kab mob (mob ntsws, sepsis), tshuaj tua kab mob thiab noj cov tshuaj. Intravenously muab ntshav, tshwj xeeb yog leukocyte loj. Thaum lub cev agranulocytosis qhia hormonal tshuaj. Cov no muaj xws "hydrocortisone" tshuaj "Prednisolone". Yog hais tias lub syndrome yog tshwm sim los ntawm inhibition ntawm hematopoiesis, stimulatory neeg sawv cev yog muab. Ntawm lawv yog cov cov tshuaj "Granocyte", "leucogen", "Virudan". Kev kho mob yog nqa tawm nyob rau hauv lub hematology department.

Kev tiv thaiv ntawm agranulocytosis nyob rau hauv cov me nyuam thiab cov laus

Kev tiv thaiv ntawm congenital agranulocytosis muaj xws li thaum ntxov ntawm tus kab mob. Yuav kom qhov no kawg, cov me nyuam ua CBC txhua xyoo. Los ntawm kev tiv thaiv kev ntsuas muaj xws li xyuas ntawm cov neeg mob uas tau txais cytotoxic tshuaj, anti-mob ntshav qab zib rau cov tshuaj. Yog hais tias ib tug neeg mob tau yav tas los tau cai Hematological lo lus teb rau thawj coj ntawm tej yam tshuaj, cov tshuaj no tsis tau reappointed.

Huab cua rau lub neej nrog agranulocytosis

Cov raug nyob rau qhov ua rau ntawm lub syndrome. Cov feem ntau txaus ntshai hauv daim ntawv ntawm ib tug hapten agranulocytosis, vim nws yog sai tsim cov ntaub so ntswg necrosis. Autoimmune thiab myelotoxic kev puas tsuaj kho tau ua tsaug rau lub hloov txoj kev kho. Tsis raug cai nyob rau hauv txoj kev loj hlob ntawm cov teeb meem ntawm lub qhov pib pathology. Cov lawv - lub loj heev los ntshav, perforation ntawm lub plob tsis so tswj phab ntsa, hemorrhagic mob ntsws, sepsis.