造血系統(tǒng)疾病課件

造血系統(tǒng)疾病1Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Diseases

of

the

Blood

System小兒造血與血液特點(diǎn)2Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.The

pediatric

characteristics

ofhematopiesis

and

blood

filmHemopoiesis3Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Definition

：Blood

cells

are

short-lived,

and

theymust

be

continually replaced

by

new

cellsformed

in

the

generative

process

calledhemopoiesis.Features

of

hematopoiesisin

children胚胎期造血Hematopoiesis

in

embryonic

period生后造血Hematopoiesis

after

birth4Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.胚胎期造血Hematopoiesis

in

embryonic

period中胚葉造血

肝（脾）造血骨髓造血5Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.6第10-14天卵黃囊間充質(zhì)C血島(blood

island)中央C

周邊C造血干C

內(nèi)皮C血C內(nèi)皮管(原始血管)中胚葉造血期Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.7肝（脾）造血期8Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.肝：胚胎

6～8周─→24周┄→（紅、粒）脾：胚胎

8周─→20周（紅、粒）胚胎

12周─→終生（淋巴、單核）胸腺：胚胎

6～7周─→終生（淋巴）淋巴結(jié)：胚胎

11周─→終生（淋巴）骨髓造血期9Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.時(shí)間：胎齡20～24周─→終生中胚葉10Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.生后造血11Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Hematopoiesis

in

after-born骨髓造血淋巴器官造血單核巨噬細(xì)胞造血骨髓外造血Hematopoesis

in

bone

marrowRed

bone

marrowhematopoiesisYellow

bone

marrow

is

deficiency

in

children,

especially

ininfant

and

toddler

period.Red

marrow Yellow

bone

marrow

(5-7yrs)Yellow

bone

marrow

can

come

back

to

red

bone

marrowwhen

hematopoetic

need

increases

.12Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.造血干細(xì)胞→造血祖細(xì)胞→

原始血細(xì)胞→幼稚血細(xì)胞→成熟血細(xì)胞13Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.生后造血The

bone

marrow

containspluripotent

stem

cells

(CD34

cells)that

develop

into

all

the

varioustypes

of

blood

cells.14Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.stemcellHEMATOPOIESIS Lineages

forGranulocyteLymphocyteMonocytePlatelet1s5RBCLymphopoiesisMonocytopoiesisGranulopoiesisErythropoiesisThrombopoiesisLymphoblastMonoblastMyeloblastMyelocyteMetamylelocyteBand

granulocytePro-MyelocytePro-erythroblastBasophilicerythroblastPolychromaticerythroblastOrthocromaticerythroblastReticulocyteMegakaryoblastMegakaryocytePluripotent

stemcell(Hemocytoblast)骨髓外造血16Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.extramedullary

hematopoiesis——嬰幼兒時(shí)期，當(dāng)因感染、溶血、失血等原因發(fā)生貧血時(shí)，骨髓以外的組織和臟器（肝、脾、淋巴結(jié)）恢復(fù)到胎兒時(shí)期的造血狀態(tài)以增加造血滿足機(jī)體需要，臨床可表現(xiàn)為肝、脾、淋巴結(jié)腫大，周圍血中出現(xiàn)有核紅細(xì)胞及幼稚粒細(xì)胞。血象特點(diǎn)17Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Character

of

hemogramplasmafat

globuleschemical

substancescarbohydrates

proteins

hormonesgasesoxygen,

carbon

dioxide,

nitrogenblood

cellsred

blood

cellswhite

blood

cellsplatelets18Red

blood

cell

system19Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.20Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,

Inc21.RBC

is 5.0-7.0x1012/lHb

is 150-220g/lAt

birth22Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.4.3-5.4average5

x

10123.8-4.84.2

x

10125.0-7.04.0-4.3Menwomennewborninfancychild4.0-4.523Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.redminimum24Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.cell

and

hemoglobin

reaches

itsat

2-3months

of

age,

during

thisperiod,

the

red

cell

count

and

hemoglobinvaluesdrop

to

their

lowest

values

of

3x10

12

/l

and100g/l

,

respectively.

These

are

termed“physiologic

anemia”.生理性貧血Physiologic

anemia生理性貧血

Physiologic

anemiaO2↑（出生后）EPO↓骨髓造血↓胎兒紅細(xì)胞大，壽命短紅細(xì)胞破壞↑生長發(fā)育快血容量↑25Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.26Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.the

first three

days:

reticulocyte

count

isabout

4－6%

of

total

red

cellsthe

end

of

the

first

week:

It

dropsrapidlybelow

2%

(0.3-1%)at

4-6weeks:

the

value

is

increased

to

2-8%.5

months

of

age

to

adulthood

:

the

valueremains

0.5-2%.reticulocyte27Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.hemoglobina28Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.They

can

be

classified

asembryonic

:

Gower-I,

Gower-II,

Portlandfetal

:adultHbFHbA1

andHbA2hemoglobin29Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.30hemoglobin31Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.At

birth32Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.1

year2

yearWhite

blood

cell

system33Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Leukocytes

include granulocytes

,monocytes,

and

lymphocytes.granulocyteseosinophilbasophilneutrophil34Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.白細(xì)胞總數(shù)的變化35Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.%8060402004－6d

4－6yDifferential

count

of

WBC(白細(xì)胞分類計(jì)數(shù))Lymphocyte淋巴細(xì)胞Granulocyte粒細(xì)胞36Platelet

system37Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.血小板（PLT）變化不大

(150－250×109/L)38Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.PLATELETSIf

a

blood

vessel

is

cut,

platelets

stick

tothe

edges

of

the

cut

and

to

oneanother,

forming

a

plug

that

stopsbleeding.They

then

release

chemicals

that

reactwith

fibrinogen

and

other

clottingproteins,

leading

to

the

formation

of

ablood

clot.The

blood

vessel

can

then

heal

over

thecut

area.39Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Blood

volume40Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.年齡越小,血容量占體重比例越大新生兒血容量占10%

體重兒童血容量占8－10%體重41Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Blood

volume小結(jié)42Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.“骨髓外造血”是小兒造血的重要特點(diǎn)嬰兒期可出現(xiàn)“生理性貧血”嬰兒期血紅蛋白種類與成人不同白細(xì)胞分類有兩個(gè)交叉出生時(shí)高RBC、Hb和WBCMosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Anemia43Definition外周血中單位容積內(nèi)紅細(xì)胞、血紅蛋白或紅細(xì)胞壓積低于正常Anemia

is

defined

as

a

reduction

of

the

redblood

cell

volume hemoglobin

concentration

orhematocrit

below

the

range

of

values

occurringin

health

person

.44Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Introduction

of

anemiaAnemia

is

a

syndrome,

not

a

name

of

a

diseaseAnemia

is

a

common

symptoms

inmanydiseasesIn

anemia

status,

Hb

RBC

and

hematocrit

arelower

than

normalDecreases

of

Hb

is

more

important

in

judgedegree

of

anemia45Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.neonate1-4

m4-6

m6m-6

y6-14y46Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Standard

of

the

diagnosis

(參照WHO標(biāo)準(zhǔn))診斷標(biāo)準(zhǔn)Hb

g/L<145<90<100<110<120分度

Grader

（

Hb

g/L）輕度中度重度極重度neonate144～120

～90～60<60child60～9030～60<3090～120(>6y)～110(6m-6y)47Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Red

blood

cell

indicesMCHMean

Corpuscular

HemoglobinIndicates

the

hemoglobin

content

per

cell.MCV

Mean

Corpuscular

VolumeIndicates

the

size

of

RBCMCHC

Htc×10

RBCHb

×

10

RBCMean

Corpuscular

Hemoglobin

ConcentrationIndicates

the

Hb

content

per

volume

of

RBCHb

×

10Htc48Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.AnemiaMCV(fl)(80-94)MCH(pg)(28-32)MCHC(%)(32-38)Macrocytic>94>3232-38Normocytic80-9428-3232-38Microcytic<80<2832-38Hypochromic<80<28<32microcytic形態(tài)學(xué)分類

morphologic

classification49Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Blood

smear(血涂片)50Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Blood

routineHypochromic

microcytic

Macrocytic（Hb↓＞RBC↓）

（Hb↓＜RBC↓)Normocytic（Hb↓

=RBC↓）IDAmegaloblasticacute

bleedingThalassemiaVit

B12↓↓hemolytic

anemiasideroblastic

anemiafolic

acid

↓aplastic

anemiaChronic

infectionmost

of

secondaryanemia51病因?qū)W分類causal

classification紅細(xì)胞和血紅蛋白生成不足貧血RBC

and

Hb

product

insufficient

anemia溶血性貧血Hemolytic

anemia失血性貧血Blood

loss

anemia52Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.紅細(xì)胞和血紅蛋白生成不足缺乏造血物質(zhì)：如鐵缺乏造成缺鐵性貧血，維生素B12，葉酸缺乏造成營養(yǎng)性巨幼細(xì)胞性貧血，還有維生素B6缺乏性貧血，維生素C缺乏。骨髓造血功能障礙再生障礙性貧血：先天性如范可尼貧血，后天性有原發(fā)性和繼發(fā)生。單純紅細(xì)胞再生障礙性貧血：先天性和獲得性。紅細(xì)胞生成素不足感染性及炎癥性貧血：慢性感染（如結(jié)核?。﹥和愶L(fēng)濕病等。其他：鉛中毒、慢性腎炎、白血病等53Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.紅細(xì)胞內(nèi)在原因紅細(xì)胞外在原因溶血膜：球形紅細(xì)胞增多癥酶：葡萄糖6-磷酸脫氫酶（G-6-PD缺乏血紅蛋白：珠蛋白生成障礙性貧血（地中海貧血）自身免疫性溶血性貧血

藥物性免疫性溶血性貧血同種免疫性溶血性貧血

血型不合輸血引起的溶血感染因素物理因素化學(xué)因素動、植物因素免疫性非免疫性其他溶血性貧血： 脾功能亢進(jìn)54G6PD

Deficiency

AnemiaG-6-PD

deficiency

is

aninheritable,

X-linkedrecessive

disorder

whoseprimary

effect

is

thereduction

of

the

enzyme

G-6-PD

in

red

blood

cells,causing

destruction

of

thecells,

called

hemolysis.Ultimately,

this

hemolysisleads

to

anemia55Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Sickle

cell

anemia

is

an

inheritedcondition

which

results

in

someerythrocytes

being

malformed.The

gene

for

this

conditioncauses

the

hemoglobin

to

beincorrectly

formed,

which

in

turncauses

some

erythrocytes

totakeon

a

crescent

shape.

Thesecellsare

not

able

to

carryadequateamounts

of

oxygen

to

cells.Sickle

cell

anemia56Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Spherocytosis,

Hereditaryspherocytosis

is

afamilial

hemolyticdisorder.

HSerythrocytes

arecaused

by

membraneprotein

defectsresulting

incytoskeleton

instability57Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.失血性貧血急性失血：如創(chuàng)傷性大出血、出血性疾病慢性失血：如潰瘍病、鉤蟲病、腸息肉58Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,59Inc.臨床表現(xiàn)Clinical

manifestations一般表現(xiàn)髓外造血各系統(tǒng)表現(xiàn)蒼白、疲倦 發(fā)育落后肝、脾腫大循環(huán)和呼吸系統(tǒng)神經(jīng)系統(tǒng)消化系統(tǒng)6060osby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.循環(huán)和呼吸系統(tǒng)兩個(gè)系統(tǒng)的癥狀互相聯(lián)系代償貧血組織缺氧心率加快

呼吸加速

脈搏加強(qiáng)

動脈壓增高代償功能失調(diào)重度貧血心臟擴(kuò)大心前區(qū)收縮期雜音充血性心力衰竭食欲減退惡心、腹脹 、便秘偶有舌炎，舌乳頭萎縮消化系統(tǒng)胃腸蠕動及消化酶的分泌神經(jīng)系統(tǒng)精神不振、注意力不集中，性情易激動，昏厥年長兒可有頭痛、昏眩、眼前有黑點(diǎn)或耳鳴臨床表現(xiàn)61貧血診斷Diagnosis詳細(xì)問病史全面體檢

實(shí)驗(yàn)室檢測history

talkingphysical

examinationlaboratory

examination62Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.病史資料年齡伴隨癥狀喂養(yǎng)既往史家族史藥物、放射線貧血診斷63Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Diagnosis體格檢查生長發(fā)育

營養(yǎng)狀況

皮膚、黏膜指甲、毛發(fā)肝脾淋巴結(jié)貧血診斷64Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Diagnosis實(shí)驗(yàn)室檢查紅細(xì)胞形態(tài)網(wǎng)織紅細(xì)胞各種紅細(xì)胞指數(shù)+白細(xì)胞計(jì)數(shù)→特檢血小板計(jì)數(shù)骨髓涂片血清鐵、鐵蛋白等紅細(xì)胞脆性試驗(yàn)

血紅蛋白的分析

紅細(xì)胞酶活性抗人球蛋白試驗(yàn)貧血診斷65Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Diagnosis常用貧血實(shí)驗(yàn)室檢查步驟二系以上異常僅

Hb下降血常規(guī)骨髓涂片再生障礙性貧血白血病其它網(wǎng)織紅

+ 糞OB溶血性營養(yǎng)性出血性+↑—66貧血診斷(三部曲)病

史體格檢查 外周血象有無貧血及程度RBC形態(tài)＋Ret計(jì)數(shù)＋WBC+BPC大致原因67A.遺傳性球形細(xì)胞增多癥B.β地中海貧血C.橢圓形細(xì)胞增多癥D.口形細(xì)胞增多癥68營養(yǎng)性溶血性失血性造血性確定診斷大致原因脆性、酶學(xué)

Hb分析喂養(yǎng)史、生化治療反應(yīng)部位、凝血骨髓涂片地中海貧血

G-6-PD缺鐵性貧血巨幼細(xì)胞貧血失血性貧血再生障礙性貧血白血病69治療原則去除病因一般療法藥物治療輸血療法造血干細(xì)胞移植治療并發(fā)癥70Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,

Inc.小結(jié)貧血為綜合征應(yīng)積極尋找病因病因分為三大類不同年齡組貧血診斷標(biāo)準(zhǔn)不同診斷分三步進(jìn)行治療按六大原則71營養(yǎng)性巨幼紅細(xì)胞性貧血72Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Nutritional Megaloblastic

AnemiaDefinition73Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.營養(yǎng)性巨幼紅細(xì)胞貧血是由于維生素

B12.或（和）葉酸缺乏所引起的一種大細(xì)胞性貧血.臨床特點(diǎn)：貧血神經(jīng)精神癥狀

RBC的胞體變大骨髓中出現(xiàn)巨幼RBC用維生素B12或（和）葉酸治療有效Definition74Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Etiology吸收和運(yùn)輸障礙胃血液VB12

+糖蛋白B12—糖蛋白復(fù)合物＋轉(zhuǎn)鈷蛋白肝臟需要量增加維生素B12缺乏儲存和攝入不足

孕婦缺乏

(胎兒可通過胎盤獲得)未及時(shí)添加輔食偏食 動物性食物含VitB12豐富75Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Vitamin

B12

absorption76Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Megaloblastic

anemias–Vitamin

B12Is

found

in

meats,

eggs,

dairy

products,

and

liver.Is

absorbed

via

a

binding

glycoprotein called

intrinsic

factor(IF)Vitamin

B12

and

IF

bind

to

mucosal

cells

in

the

ileum

and

B12enters.When

B12

is

released

from

the

mucosal

cell,

it

binds

totransport

proteins

in

the

bloodstream

(transcobalamineI

III).

Type

II

is

the

primary

transport

protein.

Thereforea

congenital

deficiency

in

type

II

can

lead

to

amegaloblastic

anemia.B12

is

transported

to

the

bone

marrow

for

use

or

to

the

liverfor

storage.77Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.遺傳性葉酸代謝障礙葉酸缺乏攝入不足羊乳含葉酸量很低，牛乳經(jīng)加熱而破壞藥物作用長期服廣譜抗生素，抗癲癇藥吸收不良慢性腹瀉代謝障礙Etiology78Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Megaloblastic

anemiasFolic

acid

(pteroyl

glutamic

acid)Is

found

in

most

foods,

including

eggs,

milk,yeast,

and

liver.Is

abundant

in

green,

leafy

vegetables

andissynthesized

by

many

microorganisms.Is

destroyed

byheatAbsorption

occurs

in

the

small

intestine79Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.How

is

folic

and

B12

acid

involved

in

DNAsynthesis?80Mosby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.四氫葉酸↓核苷酸尤其胸苷合成↓DNA合成↓

RNA合成正常VitB12↓神經(jīng)髓鞘中脂蛋白合成不足細(xì)胞分裂↓細(xì)胞增殖↓蛋白質(zhì),如Hb合成正常神經(jīng)纖維功能不完整細(xì)胞巨幼變

細(xì)胞數(shù)量下降表情呆滯,反應(yīng)差發(fā)育落后,常倒退肢體震顫可有病理反射RBC↓

WBC↓

PLT↓其它組織細(xì)胞↓精神神經(jīng)癥狀巨幼細(xì)胞性貧血葉酸還原酶葉酸↓Pathogenesis81一般表現(xiàn)皮膚蠟黃, 結(jié)膜、唇、指甲蒼白, 虛胖，頭發(fā)稀疏、枯黃, 重者皮下出血髓外造血肝、脾腫大神經(jīng)精神癥狀煩躁不安，易怒Ｂ12缺乏（神經(jīng)系統(tǒng)癥狀）遲鈍、呆滯、嗜睡、少哭不笑，智力、動作發(fā)育落后/退步,震顫, 嚴(yán)重者抽搐、感覺異常、共濟(jì)失調(diào)葉酸缺乏 可導(dǎo)致神經(jīng)精神異常，但不發(fā)生神經(jīng)系統(tǒng)癥狀消化系統(tǒng)癥狀納差、 惡心、嘔吐、腹瀉、舌M炎o(hù)sby

items

and

derived

items

?

2005,

2001

by

Mosby,Inc.Clinical

manifestations82Laboratory

examination血象RBC↓↓

Hb↓大細(xì)胞性貧血白細(xì)胞、血小板計(jì)數(shù)常減少網(wǎng)織RBC↓MCV↑(>94

fl)、MCH↑(>32pg)血涂片可見RBC大小不等，以大細(xì)胞為多易見嗜多色性和嗜堿點(diǎn)彩RB