血常規(guī)的兒科臨床實(shí)踐教學(xué)中的解讀

1、Ding ZhouzhiDepartment of Pediatrics Bengbu Medical College,血常規(guī)在兒科臨床實(shí)踐教學(xué)中的解讀,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),2,CBC……Blood Routine,Red Blood Cell……RBCHemoglubin……HbRed blood cell indices:MCV,MCH,MCHCHematocrit……HCT Reticul

2、ocyte……RetWhite Blood Cell……WBC, leukocyteWhite blood cell types ……WBC differentialNeutrophils, Lymphocytes, Monocytes, Eosinophils, and BasophilsPlatelet (thrombocyte) ……PLT, BPCMean platelet volume ……MPV,2024/3/20

3、,兒科學(xué)教研室.臨床實(shí)踐教學(xué),3,Why It Is Done,Find the cause of symptoms such as fatigue, weakness, fever, bruising, or weight loss. Check for anemia.See how much blood has been lost if there is bleeding.Diagnose polycythemia o

4、r Check for an infection.Diagnose diseases of the blood, such as leukemia.Check how the body is dealing with some types of drug or radiation treatment.Check how abnormal bleeding is affecting the blood cells and

5、counts.Screen for high and low values before a surgery.See if there are too many or too few of certain types of cells. This may help find other conditions, such as too many eosinophils may mean an allergy or&

6、#160;asthma is present.,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),4,RBC & Hb,正常值隨著年齡的變化而變化。,2024/3/20,4,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),5,RBC & Hb,正常值隨著年齡的變化而變化。,2024/3/20,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),6,RBC & Hb,2024/3/20,6,2024/3/20,

7、兒科學(xué)教研室.臨床實(shí)踐教學(xué),7,2024/3/20,7,生后紅細(xì)胞計(jì)數(shù)變化,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),8,2024/3/20,8,生后Hb的變化 The change of the level of postnatal hemoglobin,,回目錄2,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),9,紅細(xì)胞增多癥,紅細(xì)胞數(shù)目、血紅蛋白、紅細(xì)胞壓積和血液總?cè)萘匡@著地超過正常水平。新生兒期RBC≥7.0

8、5;109/L，Hb≥220g/L。兒童時(shí)期RBC≥5.0×109/L，Hb≥180g/L(16g/dl)，紅細(xì)胞壓積大于55%和每公斤體重紅細(xì)胞容量絕對(duì)值超過35ml，排除因急性脫水或燒傷等所致的血液濃縮而發(fā)生的相對(duì)性紅細(xì)胞增多，即可診斷?！　≡l(fā)性的即真性紅細(xì)胞增多癥繼發(fā)性的主要是由組織缺氧所引起的。,2024/3/20,9,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),10,2024/3/20,10,最低標(biāo)準(zhǔn) Hb

9、 minimum standard in normal children,,* ---- WHO標(biāo)準(zhǔn)(1972)# ---- 聯(lián)合國兒童基金會(huì)標(biāo)準(zhǔn)(1986)**---- 國內(nèi)標(biāo)準(zhǔn)(1989)(海拔每↑1000米，相應(yīng)診斷標(biāo)準(zhǔn)中Hb↑4%),,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),11,2024/3/20,11,RBC & Hb,生理性貧血 Physiologic anemia:Physiologic

10、hemolysis Increased blood volumeTemporary hypofunction of myeloid hematopoiesis,使RBC生成不足,約2~3月時(shí): RBC降至3.0×1012/L Hb降到100g/L左右，最低9

11、0g/L,生后Hb變化,,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),12,2024/3/20,12,Graduation of anemia in children,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),13,2024/3/20,13,形態(tài)分類 Classification of morphology,貧血類型 MCV(fl) MCH(pg) MVHC(%)正常值

12、 80~94 28~32 32~38正細(xì)胞性 80~94 28~32 32~38大細(xì)胞性 >94 >32 32~38 單純小細(xì)胞性 <80 <28

13、32~38 小細(xì)胞低色素性 <80 <28 <32,,,,,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),14,2024/3/20,14,形態(tài)學(xué)與病因?qū)W的關(guān)系 (Relation between morphology and etiology),RBC形態(tài) 病因 正細(xì)胞性

14、 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,

15、鐵 粒幼性貧血.,慢性失血，鉛中毒,,,,,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),15,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。見于再生障礙性貧血，溶血性貧血

16、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。,2024/3/20,15,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),16,2024/3/20,16,Disaster of Leukecytes,Neoplastic Disorders of Leukocytes MDS; Myelodysplastic syndrom

17、e Non-Neoplastic Disorders of Leukocytes,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),17,2024/3/20,17,不同年齡的白細(xì)胞水平變化,Cord blood: 15~20×109/L6~12h: 21~28×109/L1w: 12×109/L<1y: 10×109/L≥1y:

18、 4~12×109/L,,,,Leucocyte,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),18,White blood cell types (WBC differential) in Adult,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),19,2024/3/20,19,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),20,2024/3/20,20,Neoplastic Disorders of Leuko

19、cytes,Leukemia(acute, chronic)Lymphocytic Leukemia (ALL, CLL)Non-Lymphocytic Leukemia (myelogenous leukemia)(AML, CML)LymphomaHodgkin's lymphomaNon Hodgkin lymphoma Histiocytosis (malignant, Langerhans cell)O

20、thersMultiple myelomaNeuroblastoma etc.,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),21,2024/3/20,21,Myelodysplastic syndrome,Refractory anemia (RA) :Refractory neutropenia, Refractory anemia with ringed sideroblasts (RARS) :Refractory c

21、ytopenia with multilineage dysplasiaRefractory anemia with excess blasts (RAEB) :Refractory anemia with excess blasts I and II. RAEB was divided into *RAEB-I (5-9% blasts) and RAEB-II (10-19%) blasts, which has a poorer

22、 prognosis than RAEB-I. Refractory anemia with excess blasts in transformation (RAEB-T) :characterized by 21-30% myeloblasts in the marrow Chronic myelomonocytic leukemia (put in a new category of myelodysplastic-myelo

23、proliferative overlap syndromes.),2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),22,2024/3/20,22,Non-Neoplastic Disorders of Leukocytes,Qualitative DisordersFunctional defectQuantitative DisordersLeukocytosis:Neutrophilia, Lymphocytosis, Mo

24、nocytosis, Eosinophilia, BasophiliaLeukopenia: Granulocytopenia(Agranulocytosis ), Lymphocytopenia,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),23,2024/3/20,23,Defects of Neutrophil Function,Chronic granulomatous disease (慢性肉芽腫性疾?。-linked

25、 or autosomal recessiveInability to generate H2O2Recurrent infections by catalase-positive organisms (Staphylococcus, Serratia, Salmonella)Myeloperoxidase deficiency （髓過氧物酶缺乏）Autosomal recessiveRecurrent candidal in

26、fections,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),24,2024/3/20,24,Defects of Neutrophil Function,Chediak-Higashi Syndrome（謝迪亞克—東綜合征，即白細(xì)胞異常色素減退綜合征）Autosomal recessiveFused lysosomes – giant granules in leukocytesNeutropenia, impaired c

27、hemotaxis and bactericidal activityBacterial and fungal infections, defective platelet aggregation (prolonged bleeding time), oculocutaneous albinismAccelerated phase (lymphoproliferative disorder),2024/3/20,兒科學(xué)教研室.臨床實(shí)

28、踐教學(xué),25,2024/3/20,Blood diseases in childhood,25,Chediak-Higashi syndrome – giant granules in leukocytes,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),26,2024/3/20,26,Leukocytosis,Leukocytosis is a white blood cell count (the leukocyte count)

29、above the normal range in the blood.,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),27,2024/3/20,27,Vary with age,Cord blood: 9~30×109/L6~12h: 21~28×109/L2w: 5~21×109/L6m~6y: 6~15×109/L>6y: 4

30、.5~13.5×109/L,,,,③ Leucocyte,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),28,2024/3/20,28,Blood Cell Indices During Gestation and at Birth,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),29,2024/3/20,29,Leukemoid reaction,類白血病反應(yīng)(leukemoid reaction)是指患者在某些情況下出現(xiàn)

31、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，與某些白血病相類似，但隨后病程或尸檢證實(shí)沒有白血病。類白血病反應(yīng)是正常骨髓對(duì)某些刺激信號(hào)作出的一種反應(yīng)。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),30,2024/3/20,30,類白血病反應(yīng)的病因,1.感染是最常見的原因　　常見病原體有細(xì)菌、螺旋體、原蟲、病毒等。分為以下幾類： (1)粒細(xì)胞型類白血病反應(yīng)：常見于肺炎、腦膜炎、白喉、結(jié)核病(主

32、要為粟粒性結(jié)核、浸潤性結(jié)核溶解播散期肺外結(jié)核)等重癥傳染病。 (2)淋巴細(xì)胞型類白血病反應(yīng)：常見于百日咳、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)胞型類白血病反應(yīng)：常見于結(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)胞型類白血病反應(yīng)：常見于寄生蟲感染，如血吸蟲病、絲蟲病、瘧疾、棘球蚴病（包蟲?。┑取?2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),31,2024/3/20,31

33、,類白血病反應(yīng)的病因,2.惡性腫瘤多見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白血病反應(yīng)多屬粒細(xì)胞型亦有類似紅白血病，淋巴細(xì)胞型較少見并常伴有貧血和血小板減少。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),32,2024/3/20,32,類白血病反應(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)

34、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。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),33,2024/3/20,33,類白血病反應(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.急性組織損傷常見于外傷性組織創(chuàng)傷(如顱腦外傷、擠壓綜

35、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(如剝脫性皮炎過敏性肺炎)高熱中暑電離輻射性疾病脾切除術(shù)后,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),34,2024/3/20,34,類白血病反應(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,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué)

36、,35,2024/3/20,35,Neutrophilia,Neutrophil leukocytosis, Neutrophilic granulocytosis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,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),36,2024/3/20,36,Neut

37、rophilia,原因：Infections：bacteria, some virusNeoplasms: Hemorrhage, hemolysisHereditaryImmunological inflammation – rheumatoid arthritis, vasculitisDrugs – glucocorticoids, colony stimulating factors, lithiumM

38、etabolic – acidosis, uremia, goutTissue necrosis – infarction, burns, trauma, cold, hot, sport, anoxia, emotional,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),37,2024/3/20,37,中性粒細(xì)胞型類白血病反應(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

39、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，沒有白血病細(xì)胞的形態(tài)異常,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),38,2024/3/20,38,Lymphocytosis,Absolute lymphocytosis：the absolute lymphocyte countAdult:> 4×109/LOlder children

40、> 7×109/LInfants 9×109/LRelative lymphocytosis Adult:> 40%Children:,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),39,2024/3/20,39,Cause of Lymphocytosis,Infections:Acute infection:Infectious mononucleosis, infectious lymp

41、hocytosis, hepatitis and CMV, pertussis(百日咳) ，chicken poxChronic infection: TB, Brucellosis(布氏桿菌病 ) Some protozoal infections: toxoplasmosis and American trypanosomiasis (錐蟲病) Post-Splenectomy StateAn autoimmune diso

42、rder causing ongoing (chronic) inflammation：Crohn‘s disease，Ulcerative colitis，Vasculitis,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),40,2024/3/20,40,淋巴細(xì)胞型類白血病反應(yīng),白細(xì)胞數(shù)明顯增多，超過50×109/L，其中40%以上為淋巴細(xì)胞；若白細(xì)胞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，沒有白血病細(xì)胞的

43、形態(tài)異常,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),41,2024/3/20,41,Monocytosis,>0.95×109/L 或比值明顯升高Causes:Chronic Inflammation：Infections:tuberculosis, brucellosis, listeriosis, subacute bacterial endocarditis, syphilis, and othe

44、r viral infections and many protozoal and rickettsial infections (e.g. kala azar, malaria). Immunologic disorders: Autoimmune diseases and vasculitis chronic neutropenia and myeloproliferative disorders.,2024/3/20,兒科學(xué)教

45、研室.臨床實(shí)踐教學(xué),42,2024/3/20,42,單核細(xì)胞型類白血病反應(yīng),白細(xì)胞>30×l09/L，單核細(xì)胞>30%；若白細(xì)胞5%；骨髓象除了有單核細(xì)胞增生外，沒有白血病細(xì)胞的形態(tài)異常,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),43,2024/3/20,43,Eosinophilia,>0.45×109/L  Causes:Allergic disorders Parasi

46、tic infectionsSome forms of malignancySystemic autoimmune diseases(e.g. SLE) Some forms of vasculitis Coccidioidomycosis (球孢子菌病 ), Interstitial nephropathy Hyperimmunoglobulin E syndrome,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),44,

47、2024/3/20,44,嗜酸粒細(xì)胞型類白血病反應(yīng),外周血嗜酸性粒細(xì)胞明顯增加但無幼稚嗜酸粒細(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)無異常,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),45,2024/3/20,45,特發(fā)性嗜酸粒細(xì)胞增多綜合征 Idiopathic hypereosinophilic syndrome,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)月以上而無明顯病因的一種疾病。主要損害

48、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)的松和羥基脲無效，可選擇其他各種藥物，或白細(xì)胞去除術(shù)。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),46,2024/3/20,46,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。見于服用了大

49、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)。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),47,2024/3/20,47,Basophilia,>0.2×109/LAllergic disordersInfection – viral, TBInflammation – juvenile rheumatoid arthritis,

50、ulcerative colitisNeoplasms – chronic myeloproliferative disorders, basophilic leukemia, carcinomaEndocrine – diabetes mellitus, myxedema, estrogen therapy,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),48,2024/3/20,48,紅白血病型類白血病反應(yīng),外周血白細(xì)胞及有核紅

51、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)2%骨髓中除粒細(xì)胞系增生外，尚有紅細(xì)胞系增生,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),49,2024/3/20,49,漿細(xì)胞型類白血病反應(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，無原始細(xì)胞明顯增生等白血病征象,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),50,2024/3/20,50,Leukopenia,外周血白細(xì)胞

52、絕對(duì)計(jì)數(shù)持續(xù)低于4.0×109/L Granulopenia：Neutropenia，eosinopenia，basopeniaLymphocytopenia,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),51,2024/3/20,51,Neutropenia,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)

53、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 ),2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),52,2024/3/20,52,發(fā)病機(jī)制,①粒細(xì)胞生成減少或無效生成②粒細(xì)胞破壞喪失過多，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。,2024/3/20,兒科學(xué)教研室.臨床

54、實(shí)踐教學(xué),53,2024/3/20,53,Causes of Neutropenia,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；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

55、、布魯菌病、粟粒性結(jié)核；病毒感染，如肝炎、艾滋病等；⑦血液病：如骨髓轉(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,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),54,2024/3/20,54,Causes of Neutropenia,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

56、常綜合征、陣發(fā)性睡眠性血紅蛋白尿癥等,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),55,2024/3/20,55,Causes of Neutropenia,2. 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。,2024

57、/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),56,2024/3/20,56,Causes of Neutropenia,3.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、過敏性疾患等。 　(2)破壞增多：如脾功能亢進(jìn)等。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),57,2024/3/20,57,Treatmen of Neutropenia,病因治療抗感染升中性粒細(xì)胞數(shù)的治療 骨髓移植,2024/

58、3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),58,2024/3/20,58,Treatmen of Neutropenia,抗感染只有發(fā)熱而無膿毒血癥表現(xiàn)者，盡量在門診治療以避免醫(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,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),59,2024/3/20,

59、59,Treatmen of Neutropenia,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,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),60,2024/3/20,

60、60,Treatmen of Neutropenia,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)胞抗體陽性或由

61、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。,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),61,2024/3/20,61,Lymphopenia,<1.5×109/L (adults)<3.0×109/L (children)Decreased productionImmun

62、odeficiency syndromesHodgkin lymphomaIncreased destructionDrugsRadiationAIDSLoss of lymphocytesCollagen vascular diseasesLoss of lymphIncreased central venous pressure,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),62,2024/3/20,62,

63、Blood routine,Liuhong,male,2 years old,Fever and sore throat for 2 daysRBC 3.02×1012/L Hb 92g/LWBC 11×109/LN: 0.68, L: 0.32,catalogue2,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),63,2024/3/20,63,blood platelet,100~300×

64、109/L,回目錄2,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),64,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？,,2024/3/20,64,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),65,Primary ITP,Primary ITP is an autoimmune dis

65、order characterized by isolated thrombocytopenia (peripheral blood platelet count <100×109/L) in the absence of other causes or disorders that may be associated with thrombocytopenia.歐洲仍定義為 peripheral blood plat

66、elet count <150×109/LThe diagnosis of primary ITP remains one of exclusion; no robust clinical or laboratory parameters are currently available to establish its diagnosis with accuracy. The main clinical problem

67、 of primary ITP is an increased risk of bleeding, although bleeding symptoms may not always be present.,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),66,The IWG and American Society of Hematology based their recommendations for the use of an

68、upper threshold platelet count of 100 ×109/L on three considerations:,A study demonstrating that patients presenting with a platelet count between 100 and 150×109/L have only a 6.9% chance of developing a persi

69、stent platelet count of less than 100×109/L over 10 years of follow-up;Recognition that in non-Western ethnicities normal values in healthy individuals may be between 100 and 150×109/L, The hypothesis that a

70、cut-off value of 100×109/L would reduce concern over the mild “physiological” thrombocytopenia associated with pregnancy.,2024/3/20,兒科學(xué)教研室.臨床實(shí)踐教學(xué),67,Primary ITP,Primary ITP is an autoimmune disorder characterized by

71、 isolated thrombocytopenia (peripheral blood platelet count <100×109/L) in the absence of other causes or disorders that may be associated with thrombocytopenia.歐洲仍定義為 peripheral blood platelet count <150

72、5;109/LThe diagnosis of primary ITP remains one of exclusion; no robust clinical or laboratory parameters are currently available to establish its diagnosis with accuracy. The main clinical problem of primary ITP is an