血常規(guī)的兒科臨床實(shí)踐教學(xué)中的解讀原創(chuàng)課件

DingZhouzhi

DepartmentofPediatricsBengbuMedicalCollege血常規(guī)在兒科臨床實(shí)踐.CBC……BloodRoutineRedBloodCell……RBC

Hemoglubin……Hb

Redbloodcellindices:MCV,MCH,MCHC

Hematocrit……HCT

Reticulocyte……RetWhiteBloodCell……WBC,leukocyteWhitebloodcelltypes……WBCdifferentialNeutrophils,Lymphocytes,Monocytes,Eosinophils,andBasophilsPlatelet(thrombocyte)……PLT,BPC

Meanplateletvolume……MPV.7/22/20232兒科學(xué)教研室.臨床實(shí)踐教學(xué)WhyItIsDoneFindthecauseofsymptomssuchasfatigue,weakness,fever,bruising,orweightloss.Checkforanemia.Seehowmuchbloodhasbeenlostifthereisbleeding.Diagnose

polycythemiaorCheckforaninfection.Diagnosediseasesoftheblood,suchasleukemia.Checkhowthebodyisdealingwithsometypesofdrugor

radiationtreatment.Checkhowabnormalbleedingisaffectingthebloodcellsandcounts.Screenforhighandlowvaluesbeforeasurgery.Seeiftherearetoomanyortoofewofcertaintypesofcells.Thismayhelpfindotherconditions,suchastoomanyeosinophilsmaymeanan

allergy

or

asthma

ispresent..7/22/20233兒科學(xué)教研室.臨床實(shí)踐教學(xué)RBC&Hb正常值隨著年齡的變化而變化。7/22/20234Redbloodcell(RBC)count

Men4.5–5.5millionRBCs/mcLor4.5–5.5×1012/LWomen4.0–5.0millionRBCs/mcLor4.0–5.0×1012/LChildren3.8–6.0millionRBCs/mcLor3.8–6.0×1012/LNewborn4.1–6.1millionRBCs/mcLor4.1–6.1×1012/L.7/22/20234兒科學(xué)教研室.臨床實(shí)踐教學(xué)RBC&Hb正常值隨著年齡的變化而變化。7/22/2023Hematocrit(HCT)

Men42%–52%or0.42–0.52volumefractionWomen36%–48%or0.36–0.48volumefractionChildren29%–59%or0.29–0.59volumefractionNewborn44%–64%or0.44–0.64volumefraction.7/22/20235兒科學(xué)教研室.臨床實(shí)踐教學(xué)RBC&Hb7/22/20236Hemoglobin(Hgb)

Men14–17.4

gramsperdeciliter(g/dL)

or

140–174

gramsperliter(g/L)Women12–16g/dLor120–160g/LChildren9.5–20.5g/dLor95–205g/LNewborn14.5–24.5g/dLor145–245g/L.7/22/20236兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/20237生后紅細(xì)胞計(jì)數(shù)變化.7/22/20237兒科學(xué)教研室.臨床實(shí)踐教學(xué).7/22/20238兒科學(xué)教研室.臨床實(shí)踐教學(xué).7/22/20239兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202310最低標(biāo)準(zhǔn)Hbminimumstandardinnormalchildren年齡Neonate1~4m4~6m6m~59m5~11y12~14yHb最低值(g/L)

HCT145**90

#100

#110

0.33*1150.34*120

0.36**----WHO標(biāo)準(zhǔn)(1972)

#----聯(lián)合國(guó)兒童基金會(huì)標(biāo)準(zhǔn)(1986)

**----國(guó)內(nèi)標(biāo)準(zhǔn)(1989)(海拔每↑1000米，相應(yīng)診斷標(biāo)準(zhǔn)中Hb↑4%).7/22/202310兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202311RBC&Hb生理性貧血Physiologicanemia:PhysiologichemolysisIncreasedbloodvolumeTemporaryhypofunctionofmyeloidhematopoiesis使RBC生成不足,約2~3月時(shí):

RBC降至3.0×1012/LHb降到100g/L左右，最低90g/L生后Hb變化.7/22/202311兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202312GraduationofanemiainchildrenGraduationHbNeonateHb輕度≥90g/L≥120g/L中度≥60g/L≥90g/L重度≥30g/L≥60g/L極重度<30g/L<60g/L.7/22/202312兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202313形態(tài)分類(lèi)Classificationofmorphology貧血類(lèi)型MCV(fl)MCH(pg)MVHC(%)

正常值80~9428~3232~38

正細(xì)胞性80~9428~3232~38

大細(xì)胞性>94>3232~38

單純小細(xì)胞性<80<2832~38

小細(xì)胞低色素性<80<28<32

.7/22/202313兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202314形態(tài)學(xué)與病因?qū)W的關(guān)系

(Relationbetweenmorphologyandetiology)RBC形態(tài)病因正細(xì)胞性急性失血,溶血,再障,脾亢,腫瘤,急性感染大細(xì)胞性DNA合成障礙:Vit.B12、葉酸缺乏,幼年性

惡性貧血,藥物性貧血,紅白血病單純小細(xì)胞性缺鐵早期,慢性感染,慢性疾病小細(xì)胞低色素性Hb合成障礙:缺鐵性貧血,地中海貧血,鐵

粒幼性貧血.,慢性失血，鉛中毒.7/22/202314兒科學(xué)教研室.臨床實(shí)踐教學(xué)網(wǎng)織紅細(xì)胞的意義網(wǎng)織紅細(xì)胞增多：表示骨髓紅細(xì)胞生成旺盛，常見(jiàn)于溶血性貧血，特別是急性溶血（高達(dá)0.6～0.8）。急性失血后5～10天網(wǎng)織紅細(xì)胞達(dá)高峰，2周后恢復(fù)正常。網(wǎng)織紅細(xì)胞減少：提示骨髓增生功能低下。見(jiàn)于再生障礙性貧血，溶血性貧血再生危象、藥物性骨髓抑制時(shí)，典型再生障礙性貧血，網(wǎng)織紅細(xì)胞計(jì)數(shù)常低于0.005.網(wǎng)織紅細(xì)胞絕對(duì)值低于15×109/L為再生障礙性貧血的診斷標(biāo)準(zhǔn)之一。7/22/202315.7/22/202315兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202316DisasterofLeukecytesNeoplasticDisordersofLeukocytesMDS;Myelodysplasticsyndrome

Non-NeoplasticDisordersofLeukocytes.7/22/202316兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202317不同年齡的白細(xì)胞水平變化Cordblood:15~20×109/L6~12h:21~28×109/L1w:12×109/L<1y:10×109/L≥1y:4~12×109/LLeucocyte.7/22/202317兒科學(xué)教研室.臨床實(shí)踐教學(xué)Whitebloodcelltypes(WBCdifferential)inAdultNeutrophils50%–62%Bandneutrophils3%–6%Lymphocytes25%–40%Monocytes3%–7%Eosinophils0%–3%Basophils0%–1%.7/22/202318兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202319

4-6天1-4歲4-6歲7歲后80%060%40%20%時(shí)間LymphocyteNeutrophil兒科中性粒細(xì)胞和淋巴細(xì)胞比例變化.7/22/202319兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202320NeoplasticDisordersofLeukocytesLeukemia(acute,chronic)LymphocyticLeukemia(ALL,CLL)Non-LymphocyticLeukemia(myelogenousleukemia)

(AML,CML)LymphomaHodgkin'slymphomaNonHodgkinlymphoma

Histiocytosis(malignant,Langerhanscell)OthersMultiplemyelomaNeuroblastomaetc..7/22/202320兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202321MyelodysplasticsyndromeRefractoryanemia

(RA):Refractoryneutropenia,Refractoryanemiawithringedsideroblasts(RARS):RefractorycytopeniawithmultilineagedysplasiaRefractoryanemiawithexcessblasts(RAEB):RefractoryanemiawithexcessblastsIandII.RAEBwasdividedinto*RAEB-I(5-9%blasts)andRAEB-II(10-19%)blasts,whichhasapoorerprognosisthanRAEB-I.Refractoryanemiawithexcessblastsintransformation(RAEB-T):characterizedby21-30%myeloblastsinthemarrowChronicmyelomonocyticleukemia(putinanewcategoryofmyelodysplastic-myeloproliferativeoverlapsyndromes.).7/22/202321兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202322Non-NeoplasticDisordersofLeukocytesQualitativeDisordersFunctionaldefectQuantitativeDisordersLeukocytosis:Neutrophilia,Lymphocytosis,Monocytosis,Eosinophilia,BasophiliaLeukopenia:Granulocytopenia(Agranulocytosis),Lymphocytopenia.7/22/202322兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202323DefectsofNeutrophilFunctionChronicgranulomatousdisease(慢性肉芽腫性疾?。-linkedorautosomalrecessiveInabilitytogenerateH2O2Recurrentinfectionsbycatalase-positiveorganisms(Staphylococcus,Serratia,Salmonella)Myeloperoxidasedeficiency（髓過(guò)氧物酶缺乏）AutosomalrecessiveRecurrentcandidalinfections.7/22/202323兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202324DefectsofNeutrophilFunctionChediak-HigashiSyndrome（謝迪亞克—東綜合征，即白細(xì)胞異常色素減退綜合征）AutosomalrecessiveFusedlysosomes–

giantgranulesinleukocytesNeutropenia,impairedchemotaxisandbactericidalactivityBacterialandfungalinfections,defectiveplateletaggregation(prolongedbleedingtime),oculocutaneousalbinismAcceleratedphase(lymphoproliferativedisorder).7/22/202324兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/2023Blooddiseasesinchildhood25Chediak-Higashisyndrome–giantgranulesinleukocytes.7/22/202325兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202326LeukocytosisLeukocytosisisawhitebloodcellcount(theleukocytecount)abovethenormalrangeintheblood..7/22/202326兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202327VarywithageCordblood:9~30×109/L6~12h:21~28×109/L2w:5~21×109/L6m~6y:6~15×109/L>6y:4.5~13.5×109/L③Leucocyte.7/22/202327兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202328BloodCellIndicesDuringGestationandatBirth

WeekofGestationCorrectedWBCCounts(×109/L)Platelets(×109/L)RBC(×109/L)Hb(g/dL)18–21(n=760)2.57±0.42234±572.85±0.3611.69±1.2722–25(n=1,200)3.73±2.17247±593.09±0.3412.2±1.626–29(n=460)4.08±0.84242±693.46±0.4112.91±1.38>30(n=440)6.40±2.99232±873.82±0.6413.64±2.21Term18.1(9.0–30.0)290±1004.70±0.4016.5±1.5.7/22/202328兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202329Leukemoidreaction類(lèi)白血病反應(yīng)(leukemoidreaction)是指患者在某些情況下出現(xiàn)外周血白細(xì)胞顯著增高(＞50×109/L)和(或)存在有異常未成熟白細(xì)胞，與某些白血病相類(lèi)似，但隨后病程或尸檢證實(shí)沒(méi)有白血病。類(lèi)白血病反應(yīng)是正常骨髓對(duì)某些刺激信號(hào)作出的一種反應(yīng)。.7/22/202329兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202330類(lèi)白血病反應(yīng)的病因1.感染是最常見(jiàn)的原因常見(jiàn)病原體有細(xì)菌、螺旋體、原蟲(chóng)、病毒等。分為以下幾類(lèi)：(1)粒細(xì)胞型類(lèi)白血病反應(yīng)：常見(jiàn)于肺炎、腦膜炎、白喉、結(jié)核病(主要為粟粒性結(jié)核、浸潤(rùn)性結(jié)核溶解播散期肺外結(jié)核)等重癥傳染病。(2)淋巴細(xì)胞型類(lèi)白血病反應(yīng)：常見(jiàn)于百日咳、水痘、傳染性單核細(xì)胞增多癥、傳染性淋巴細(xì)胞增多癥、結(jié)核病等。(3)單核細(xì)胞型類(lèi)白血病反應(yīng)：常見(jiàn)于結(jié)核病、巨細(xì)胞病毒感染、亞急性細(xì)菌性心內(nèi)膜炎等。(4)嗜酸性粒細(xì)胞型類(lèi)白血病反應(yīng)：常見(jiàn)于寄生蟲(chóng)感染，如血吸蟲(chóng)病、絲蟲(chóng)病、瘧疾、棘球蚴?。òx(chóng)病）等。.7/22/202330兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202331類(lèi)白血病反應(yīng)的病因2.惡性腫瘤多見(jiàn)于晚期患者肺和胃腸道惡性腫瘤，尤其是轉(zhuǎn)移到肝骨髓后易發(fā)生類(lèi)白血病反應(yīng)。多發(fā)性骨髓瘤、霍奇金病、黑色素瘤骨肉瘤、乳腺癌、絨毛膜上皮癌腫瘤引起的類(lèi)白血病反應(yīng)多屬粒細(xì)胞型亦有類(lèi)似紅白血病，淋巴細(xì)胞型較少見(jiàn)并常伴有貧血和血小板減少。.7/22/202331兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202332類(lèi)白血病反應(yīng)的病因3.中毒(1)化學(xué)因素：如汞、有機(jī)磷、苯、亞硝酸鹽等中毒。(2)藥物性：如砷劑、解熱鎮(zhèn)痛藥、磺胺藥、腎上腺素糖皮質(zhì)激素、鋰鹽等。還有報(bào)道：用大劑量阿糖胞苷治療急性白血病緩解期引起的類(lèi)白血病反應(yīng)，易誤診為“復(fù)發(fā)”。(3)其他：一氧化碳中毒四氯乙烷中毒、尿毒癥、酮癥酸中毒、食物中毒等。.7/22/202332兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202333類(lèi)白血病反應(yīng)的病因4.急性失血與溶血任何原因引起的大出血、急性血管內(nèi)溶血。5.急性組織損傷常見(jiàn)于外傷性組織創(chuàng)傷(如顱腦外傷、擠壓綜合征)、大面積燒傷、電休克等。6.其他疾病變態(tài)反應(yīng)性疾病(如剝脫性皮炎過(guò)敏性肺炎)高熱中暑電離輻射性疾病脾切除術(shù)后.7/22/202333兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202334類(lèi)白血病反應(yīng)的診斷1.有明確的病因，如感染中毒、惡性腫瘤等；

2.原發(fā)病治愈或好轉(zhuǎn)后，類(lèi)白血病反應(yīng)可迅速消失；

3.血紅蛋白、血小板計(jì)數(shù)大致正常。4.骨髓檢查：反應(yīng)性增生

.7/22/202334兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202335NeutrophiliaNeutrophilleukocytosis,Neutrophilicgranulocytosis年齡大于1個(gè)月的兒童和各年齡組成人外周血中性桿狀核和分葉核粒細(xì)胞計(jì)數(shù)大于7.5×109/L小于1個(gè)月的嬰兒大于26×109/L中性粒細(xì)胞比值明顯高于同年齡兒童.7/22/202335兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202336Neutrophilia原因：Infections：bacteria,somevirus Neoplasms:Hemorrhage,hemolysis HereditaryImmunologicalinflammation–rheumatoidarthritis,vasculitisDrugs–glucocorticoids,colonystimulatingfactors,lithiumMetabolic–acidosis,uremia,goutTissuenecrosis–infarction,burns,trauma,cold,hot,sport,anoxia,emotional.7/22/202336兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202337中性粒細(xì)胞型類(lèi)白血病反應(yīng)白細(xì)胞計(jì)數(shù)>50×109/L，或外周血白細(xì)胞計(jì)數(shù)<50×109/L，但出現(xiàn)原粒、幼粒細(xì)胞成熟中性粒細(xì)胞胞質(zhì)中常出現(xiàn)中毒性顆粒和空泡堿性磷酸酶積分明顯增高骨髓象除了有粒細(xì)胞增生和左移現(xiàn)象外，沒(méi)有白血病細(xì)胞的形態(tài)異常.7/22/202337兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202338LymphocytosisAbsolutelymphocytosis：theabsolutelymphocytecountAdult:>4×109/LOlderchildren>7×109/LInfants9×109/LRelativelymphocytosisAdult:>40%Children:.7/22/202338兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202339CauseofLymphocytosisInfections:Acuteinfection:Infectiousmononucleosis,infectiouslymphocytosis,hepatitisandCMV,pertussis(百日咳)，chickenpoxChronicinfection:TB,Brucellosis(布氏桿菌病)Someprotozoalinfections:toxoplasmosisandAmericantrypanosomiasis(錐蟲(chóng)病)Post-SplenectomyStateAnautoimmunedisordercausingongoing(chronic)inflammation：Crohn‘sdisease，Ulcerativecolitis，Vasculitis.7/22/202339兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202340淋巴細(xì)胞型類(lèi)白血病反應(yīng)白細(xì)胞數(shù)明顯增多，超過(guò)50×109/L，其中40%以上為淋巴細(xì)胞；若白細(xì)胞<50×109/L，其中異形淋巴細(xì)胞應(yīng)>20%，并出現(xiàn)幼淋巴細(xì)胞；骨髓象除了有淋巴細(xì)胞增生外，沒(méi)有白血病細(xì)胞的形態(tài)異常.7/22/202340兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202341Monocytosis>0.95×109/L或比值明顯升高Causes:ChronicInflammation： Infections:tuberculosis,brucellosis,listeriosis,subacutebacterialendocarditis,syphilis,andotherviralinfectionsandmanyprotozoalandrickettsialinfections(e.g.kalaazar,malaria).Immunologicdisorders:Autoimmunediseasesandvasculitischronicneutropeniaandmyeloproliferativedisorders..7/22/202341兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202342單核細(xì)胞型類(lèi)白血病反應(yīng)白細(xì)胞>30×l09/L，單核細(xì)胞>30%；若白細(xì)胞<30×109/L，幼單核細(xì)胞應(yīng)>5%；骨髓象除了有單核細(xì)胞增生外，沒(méi)有白血病細(xì)胞的形態(tài)異常.7/22/202342兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202343Eosinophilia>0.45×109/L

Causes:AllergicdisordersParasiticinfectionsSomeformsofmalignancySystemicautoimmunediseases(e.g.SLE)SomeformsofvasculitisCoccidioidomycosis(球孢子菌病),InterstitialnephropathyHyperimmunoglobulinEsyndrome.7/22/202343兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202344嗜酸粒細(xì)胞型類(lèi)白血病反應(yīng)外周血嗜酸性粒細(xì)胞明顯增加但無(wú)幼稚嗜酸粒細(xì)胞；骨髓中原始細(xì)胞比例不增高，嗜酸性粒細(xì)胞形態(tài)無(wú)異常.7/22/202344兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202345特發(fā)性嗜酸粒細(xì)胞增多綜合征

Idiopathichypereosinophilicsyndrome血中嗜酸粒細(xì)胞數(shù)量大于1.5×109/L，并持續(xù)達(dá)6個(gè)月以上而無(wú)明顯病因的一種疾病。主要損害心、肺、肝、皮膚以及神經(jīng)系統(tǒng)。若不采取治療，80％以上的病人會(huì)在2年之內(nèi)死亡；而治療后80％以上的病人能繼續(xù)存活。治療：強(qiáng)的松或羥基脲若病人對(duì)強(qiáng)的松和羥基脲無(wú)效，可選擇其他各種藥物，或白細(xì)胞去除術(shù)。.7/22/202345兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202346嗜酸粒細(xì)胞增多-肌痛綜合征一種嗜酸粒細(xì)胞增多并伴有肌痛、壓痛、疲乏、腫脹、關(guān)節(jié)痛、咳嗽、呼吸急促、皮疹以及神經(jīng)系統(tǒng)異常的疾病。該綜合征少見(jiàn)。見(jiàn)于服用了大量色氨酸的患者停用色氨酸后可持續(xù)數(shù)周至數(shù)月，并能引起持久的神經(jīng)損害，甚至偶可導(dǎo)致死亡目前不能治愈；一般建議理療康復(fù)。.7/22/202346兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202347Basophilia>0.2×109/LAllergicdisorders Infection–viral,TBInflammation–juvenilerheumatoidarthritis,ulcerativecolitisNeoplasms–

chronicmyeloproliferativedisorders,basophilicleukemia,carcinomaEndocrine–diabetesmellitus,myxedema,estrogentherapy.7/22/202347兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202348紅白血病型類(lèi)白血病反應(yīng)外周血白細(xì)胞及有核紅細(xì)胞總數(shù)>50×109/L并有幼稚粒細(xì)胞；若白細(xì)胞總數(shù)<50×109/L，原粒細(xì)胞應(yīng)>2%骨髓中除粒細(xì)胞系增生外，尚有紅細(xì)胞系增生.7/22/202348兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202349漿細(xì)胞型類(lèi)白血病反應(yīng)白細(xì)胞總數(shù)增多或不增多，外周血漿細(xì)胞>2%骨髓中除漿細(xì)胞系增生外，無(wú)原始細(xì)胞明顯增生等白血病征象.7/22/202349兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202350Leukopenia外周血白細(xì)胞絕對(duì)計(jì)數(shù)持續(xù)低于4.0×109/LGranulopenia：Neutropenia，eosinopenia，basopeniaLymphocytopenia.7/22/202350兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202351Neutropenia中性粒細(xì)胞減少癥是血循環(huán)池中性多形核細(xì)胞(PMN)絕對(duì)值<1.5×109/L(兒童期)，或<1.0×109/L(嬰兒期)時(shí)所出現(xiàn)的一組綜合征。輕型(1.0~1.5×109/L)中型(0.5~1.0×109/L)重型(<0.5×109/L)(Agranulocytosis).7/22/202351兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202352發(fā)病機(jī)制①粒細(xì)胞生成減少或無(wú)效生成②粒細(xì)胞破壞喪失過(guò)多，粒細(xì)胞壽命縮短③粒細(xì)胞分布異常④綜合前3種機(jī)制。.7/22/202352兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202353CausesofNeutropenia骨髓(1)骨髓損傷：①藥物：包括細(xì)胞毒和非細(xì)胞毒藥物；②放射線(xiàn)；③化學(xué)物質(zhì)：如苯、DDT、二硝基苯酚、砷酸、鉍、一氧化氮等；④某些先天性和遺傳性中性粒細(xì)胞減少：如Kostmann綜合征、伴先天性白細(xì)胞缺乏的網(wǎng)狀發(fā)育不全、伴粒細(xì)胞生成異常的中性粒細(xì)胞減少等；⑤免疫性疾患：如系統(tǒng)性紅斑狼瘡、類(lèi)風(fēng)濕性關(guān)節(jié)炎等；⑥感染：細(xì)菌性感染，如傷寒、副傷寒、布魯菌病、粟粒性結(jié)核；病毒感染，如肝炎、艾滋病等；⑦血液?。喝绻撬柁D(zhuǎn)移瘤、骨髓纖維化、淋巴瘤、白細(xì)胞減少的白血病、再生障礙性貧血、多發(fā)性骨髓瘤、惡性組織細(xì)胞增生癥等.7/22/202353兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202354CausesofNeutropenia骨髓(2)成熟障礙：①獲得性：如葉酸缺乏、維生素B12缺乏、惡性貧血、嚴(yán)重的缺鐵性貧血等；②惡性和其他克隆性疾病：如骨髓增生異常綜合征、陣發(fā)性睡眠性血紅蛋白尿癥等.7/22/202354兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202355CausesofNeutropenia2.外周血(1)中性粒細(xì)胞外循環(huán)池轉(zhuǎn)換至邊緣池(即假性中性粒細(xì)胞減少)：①遺傳性良性假性中性粒細(xì)胞減少癥；②獲得性：如嚴(yán)重鶒的細(xì)菌感染，惡性營(yíng)養(yǎng)不良病，瘧疾等。(2)血管內(nèi)扣留：如由補(bǔ)體介導(dǎo)的白細(xì)胞凝集素所致的肺內(nèi)扣留、脾功能亢進(jìn)所致的脾內(nèi)扣留等。.7/22/202355兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202356CausesofNeutropenia3.作用于血管外(1)利用增多鶒：如嚴(yán)重的細(xì)菌、真菌、病毒或立克次體感染、過(guò)敏性疾患等。(2)破壞增多：如脾功能亢進(jìn)等。.7/22/202356兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202357TreatmenofNeutropenia病因治療抗感染升中性粒細(xì)胞數(shù)的治療骨髓移植.7/22/202357兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202358TreatmenofNeutropenia抗感染只有發(fā)熱而無(wú)膿毒血癥表現(xiàn)者，盡量在門(mén)診治療以避免醫(yī)院內(nèi)繼發(fā)感染嚴(yán)重中性粒細(xì)胞減少患者出現(xiàn)發(fā)熱時(shí)，應(yīng)以急診患者對(duì)待，立即收入院治療，有條件時(shí)應(yīng)予逆向隔離。在進(jìn)行皮膚、咽喉、血、尿、大便等部位的病菌培養(yǎng)檢查后，立即給予經(jīng)驗(yàn)性廣譜抗生素治療.7/22/202358兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202359TreatmenofNeutropenia抗感染若病原菌明確患者，則根據(jù)藥敏試驗(yàn)改用針對(duì)性的窄譜抗生素若未發(fā)現(xiàn)病原菌，但經(jīng)治療后病情得以控制者在病情治愈后仍應(yīng)繼續(xù)給予口服抗生素7～14天若未發(fā)現(xiàn)病原菌，且經(jīng)前述處理3天后病情無(wú)好轉(zhuǎn)，對(duì)病情較輕者可停用經(jīng)驗(yàn)性抗生素治療，再次進(jìn)行病原菌培養(yǎng)，若病情較重者應(yīng)在原有治療基礎(chǔ)上加用抗真菌藥，如兩性霉素B等.7/22/202359兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202360TreatmenofNeutropenia升中性粒細(xì)胞數(shù)的治療(1)促白細(xì)胞生成藥：目前在臨床上應(yīng)用的很多，如維生素B6、維生素B4、利血生、肌苷、脫氧核苷酸、雄激素、碳酸鋰等，但均缺乏肯定和持久的療效，因此，初治患者可選用1～2種，每4～6周更換一組，直到有效，若連續(xù)數(shù)月仍不見(jiàn)效者，不必再繼續(xù)使用。(2)免疫抑制藥治療：如糖皮質(zhì)激素、硫唑嘌呤、環(huán)磷酰胺、大劑量人血丙種球蛋白輸注等，對(duì)部分患者，如抗中性粒細(xì)胞抗體陽(yáng)性或由細(xì)胞毒T細(xì)胞介導(dǎo)的骨髓衰竭患者等有效。(3)集落刺激因子治療(4)中性粒細(xì)胞輸注:由于中性粒細(xì)胞在外周血和組織中的生存期短，因此至少1次/d，連續(xù)3天方可起效。.7/22/202360兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202361Lymphopenia<1.5×109/L(adults)<3.0×109/L(children)DecreasedproductionImmunodeficiencysyndromesHodgkinlymphomaIncreaseddestructionDrugs RadiationAIDSLossoflymphocytesCollagenvasculardiseases LossoflymphIncreasedcentralvenouspressure.7/22/202361兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202362BloodroutineLiuhong,male,2yearsold,Feverandsorethroatfor2daysRBC3.02×1012/LHb92g/L

WBC11×109/L

N:0.68,L:0.32catalogue2.7/22/202362兒科學(xué)教研室.臨床實(shí)踐教學(xué)7/22/202363bloodplatelet100~300×109/L回目錄2.7/22/202363兒科學(xué)教研室.臨床實(shí)踐教學(xué)血小板增多癥原發(fā)性血小板增多癥是一種原因不明的骨髓增生性疾病，本病的主要特點(diǎn)是外周血中血高，伴有出血傾向，血栓形成，肝脾腫大和粒細(xì)胞增多等。到底達(dá)到多少才能診斷？到底達(dá)到多少需要治療？7/22/202364.7/22/202364兒科學(xué)教研室.臨床實(shí)踐教學(xué)PrimaryITPPrimaryITPisanautoimmunedisordercharacterizedbyisolatedthrombocytopenia(peripheralbloodplateletcount<100×109/L)intheabsenceofothercausesordisordersthatmaybeassociatedwiththrombocytopenia.歐洲仍定義為peripheralbloodplateletcount<150×109/LThediagnosisofprimaryITPremainsoneofexclusion;norobustclinicalorlaboratoryparametersarecurrentlyavailabletoestablishitsdiagnosiswithaccuracy.ThemainclinicalproblemofprimaryITPisanincreasedriskofbleeding,althoughbleedingsymptomsmaynotalwaysbepresent..7/22/202365兒科學(xué)教研室.臨床實(shí)踐教學(xué)TheIWGandAmericanSocietyofHematologybasedtheirrecommendationsfortheuseofanupperthresholdplateletcountof100×109/Lonthreeconsiderations:

Astudydemonstratingthatpatientspresentingwithaplateletcountbetween100and150×109/Lhaveonlya6.9