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1 Human Physiology - 혈액생리 (Blood) - 생리학교실이광이메일 : glee@ajou.ac.kr. 내선 : 4554 실험실 : 송재관 607 호

2 I. 혈액의기능과조성 A. 혈액의기능 1. 운반작용 : CO 2, O 2, urea, nutrients, antibody, hormones 2. 조절작용 : 혈당량, 체온, 삼투압, ph 유지 ( 생체의항상성유지 ) 3. 방어작용 : 식균작용, 혈액응고 * Self-preserve activity B. 혈액의조성 1. 세포성분 (cellular component; 45%) 1) 적혈구 ( X 10 6 / mm 3 ) 2) 백혈구 ( 과립구 (granulocytes), 무과립구 (agranulocyte); 5,000-10,000/mm 3 ) 염기성구 (basophils) % 산성구 (eosinophils) 1-3 % 중성구 (neutrophils ) % 림프구 (lymphocytes) % 단핵구 (monocytes ) 3-7 % 3) 혈소판 (platelet, thrombocytes; 140, ,000/mm 3 ) 2. 혈장성분 (plasma component, 55%) 3. 물리적특성 Hematocrit (Hct) : 전체혈액에대한적혈구의용적비율 Specific gravity (SG) : Viscosity : ca (cf. pure water : 1.00) Erythrocyte Sedimentation Rate (ESR) : 2-10 mm/h

3 혈액의구성성분 Blood is a circulating tissue consisting of three types of cells. - Red Blood Cells (Erythrocytes) - White Blood Cells (Leukocytes) - Platelets (Thrombocytes)

4 혈액의구성성분

6 혈액세포들과혈소판

Blood is red because it contains hemoglobin and iron rich pigment Plasma Plasma is the relatively clear liquid water, sugar, fat, protein and salt solution Normally, 55% of our

7 Blood is red because it contains hemoglobin and iron rich pigment Plasma Plasma is the relatively clear liquid water, sugar, fat, protein and salt solution Normally, 55% of our blood's volume is made up of plasma About 95% of it consists of water Helps to regulate body temperature Plasma transports blood cells, products of digestion and hormones throughout the body

8 Hematocrit: 적혈구용적률 polycythemia, 赤血球增加症

9 Rouleaux : 連錢狀 Red cell rouleaux are formed as shear rates are reduced and RBCs aggregate. They become jammed at the capillary entrance slowly break apart with time 진단 : 골수종, 마이크로글로부린혈증등

10 II. 혈구의생성과기능 A. 혈구의생성 1. 혈구세포의생성 1) 태아시혈액생성 (intrauterine blood formation) - 거적아세포기 {megaloblastic phase (yolk sac)}: 수정후 2개월까지난황에서거대적혈구를형성 - 간기 ( hepatic phase): 그다음 4개월까지간, 비장, 흉선, 임파절등에서혈액세포생성 - 골수기 ( myeloid phase): 출생때가지골수에서혈액세포생성 2) 출생후혈액생성 (extrauterine blood formation) - 골수성조직 (myeloid tissue) : bone marrow - 골수외혈구생성 (extramedullary hematopoiesis) : 간, 비장, 림프조직 3) 혈구의분화및증식 - Hematopoiesis; erythropoiesis, leucopoiesis, thrombocytopoiesis - Proliferation and differentiation from pluripotent, uncommitted stem cell - committed stem cell (hemocytoblast, 혈구모 ( 아 ) 세포 ) proerythroblast ( 前赤母球 ) normoblast ( 正赤母球 ) reticulocyte ( 網狀赤血球 ) erythrocyte

11 megaloblastic phase, hepatic phase, myeloid phase

12 Hematopoiesis

13 Consisting mainly hematopoietic tissue Consisting mainly of fat cells The fatty degeneration of red bone marrow (RBM) (25%) into yellow bone marrow (YBM) (75%) is completed around age 21 years.

B. 적혈구 1. 적혈구의특성 1) 형태적특성 - biconcave disc: 직경 Ca.

세포의유연성이크다 : 변형능 (deformability) - 세포막의긴장도가낮다 :

: homogenous matrix and loosely arranged meshwork of

spherocytosis ( 구상적혈구증 ), hereditary elliptocytosis

14 B. 적혈구 1. 적혈구의특성 1) 형태적특성 - biconcave disc: 직경 Ca. 8μm, 두께 1-2 μm - 표면적 / 체적비율이높다 : 세포내외로의물질확산이용이 - 세포의유연성이크다 : 변형능 (deformability) - 세포막의긴장도가낮다 : 세포용적의변화에손쉽게대응 2) 구조적특성 - 진정한의미의세포가아님 - cytoskeleton : homogenous matrix and loosely arranged meshwork of fibrous and globular proteins 3) 유전적결함 : hereditary spherocytosis ( 구상적혈구증 ), hereditary elliptocytosis ( 타원적혈구증 ) * Price-Jones curve : normal distribution of RBC size

15 Erythrocyte Deformability

16 pernicious anemia, 惡性貧血

2. 혈색소 (hemoglobin) - hemoglobin : 200-300 million molecules/rbc (34% of RBC), 분자식, C 3032 H 4816 O 872 N 780

과공유결합 3) 산소운반능력 ; 약 20 ml of oxygen/dl - 혈중에서운반되는산소의약 98 % 는 oxyhemoglobin 의형태 - 1 gram 의 hemoglobin 은 1.

17 2. 혈색소 (hemoglobin) - hemoglobin : million molecules/rbc (34% of RBC), 분자식, C 3032 H 4816 O 872 N 780 S 8 Fe 4 1) 기능 (1) 산소운반 (2) ph 변화에대한화학적인완충제 2) 구조 - 두쌍의 polypeptide 사슬 (α,β,γ,δ) 로구성된 tetramer : 각각 heme group 과공유결합 3) 산소운반능력 ; 약 20 ml of oxygen/dl - 혈중에서운반되는산소의약 98 % 는 oxyhemoglobin 의형태 - 1 gram 의 hemoglobin 은 1.34 ml 의산소와결합 4) 함량 ; 15 grams of hemoglobin/100 ml whole blood - 정상치 : 성인여성 : g/dl; 성인남성 : g/dl

18 5) hemoglobin 의종류 (1) 정상 hemoglobin; 2 types; globin 구조에차이 - Type A - Type A 1 (96%), Type A 2 (2%) - Type F (2%) (2) peptide 사슬의조성 - type A 1 : 2 α-chains, 2 β-chains - type A 2 : 2 α-chains, 2 δ-chains - type F : 2 α-chains, 2 γ-chains (3) Hemoglobin F; 태아혈액중의주성분 - O 2 에대한친화력이크다. - 출생직후 hemoglobin A 로대체 (6 개월후완전히대체 )

of oxygen dissociation curve 산화헤모글로빈해리곡선 산소의하역량 (unloading) - ph - 온도 - 2,3-BPG

19 Respiratory Function of Hemoglobin (lungs) - Deoxyhemoglobin + O2 Oxyhemoglobin (tissues) - Hemoglobin: transport O 2 and CO 2 cooperativity in the binding of oxygen sigmoid shape of oxygen dissociation curve 산화헤모글로빈해리곡선 산소의하역량 (unloading) - ph - 온도 - 2,3-BPG (2,3-biphosphoglyceric acid) 혈액이조직을통과하여동맥에서정맥으로이동 - 산화헤모글로빈의백분율이 22% 감소하여 100 ml 의혈액당 5mL 미만의산소가조직으로하역됨

20 ph 가산화헤모글로빈해리곡선에미치는영향 보어효과 (Bohr effect): 낮은 ph 에서헤모글로빈의산소친화도가떨어지는현상

21 2,3-BPG 에의해산소의하역증가 1,3-Biphosphoglycerite(BPG) 를 2,3-BPG 로변환 산소에대한 hemoglobin 의친화력감소

22 이산화탄소의수송과염소이동 carbaminohemoglobin

6) Hemoglobinopathies (1) 비정상적인 hemoglobin 의합성겸형 (

anemia Malaria Disease that attacks the RBC, if

23 6) Hemoglobinopathies (1) 비정상적인 hemoglobin 의합성겸형 ( 鎌形 ) 적혈구 : β-chain 아미노산이상 (glutamine 6 valine) sickle cell hemoglobin (type S): deoxygenation of hemoglobin S sickle shape(reversible) sickle cell anemia Malaria Disease that attacks the RBC, if you have sickle cell shaped RBC can not get the disease.

24 (2) α- or β-globin chain 합성부전 (Thalassaemia) α-thalassaemia : fatal, 동남아시아 β-thalassaemia : 지중해성빈혈 (mediterranean anemia)

25 Carbon Monoxide 일산화탄소 (Carbon monoxide, CO) - colorless - odorless - tasteless gas that is slightly less dense than air - Hemoglobin binds with CO times more readily than with oxygen

26 7) 혈색소의대사 - Hemoglobin : Heme + globin; Heme biliverdin bilirubin conjugated bilirubin mesobilirubinogen ( 장내세균에의함 ) urobilinogen, stercobilinogen 으로배설되거나재흡수 - Hyperbilirubinemia : 혈중 bilirubin 농도 2 mg/100 ml 이상 황달 (jaundice, icterus)

3. 적혈구의생성 (Erythropoiesis) - 약 230 billion RBC/ 일생성 ( 약 3 10 9 RBC s/kg/day) - 120 days 생존, 비장, 간, 골수의 macrophages에의해제거 1) 분화 (differentiation) Uncommitted stem cell (pluripotent stem cell) Committed

27 3. 적혈구의생성 (Erythropoiesis) - 약 230 billion RBC/ 일생성 ( 약 RBC s/kg/day) days 생존, 비장, 간, 골수의 macrophages에의해제거 1) 분화 (differentiation) Uncommitted stem cell (pluripotent stem cell) Committed stem cell Reticulocytes ( 망상적혈구 ): 혈행중 1.5일이내에 RBC로성숙 (maturation), 통상, RBC 의 1% 에해당 index of erythropoietic activity stains (new methylene blue) 2) Erythropoiesis 조절물질 ribosomal RNA (1) 철 - 영양요구량 성인남성및비월경여성 (nonmenstruating female) : < 1 mg / 일 menstruating female : 1.5 mg/ 일 정상식이 : mg/ 일 성장기, 임신등에의해요구량증대 - 철의흡수 : 십이지장, proximal jejunum ( 空腸 ) by phagocytosis by interaction of specific surface receptors with transferrin - 저장형 : ferritin, hemosiderin; 간및골수에각각 1/3, 나머지는비장, 근육등에저장

Erythropoiesis Stem cell Committed cell Developmental pathway Phase 1 Ribosome synthesis Phase 2 Hemoglobin accumulation Phase 3 Ejection

29 Erythropoiesis Stem cell Committed cell Developmental pathway Phase 1 Ribosome synthesis Phase 2 Hemoglobin accumulation Phase 3 Ejection of nucleus Hemocytoblast Proerythroblast Early erythroblast Late erythroblast Normoblast Reticulocyte Erythrocyte Day 1 Day2 Day4 Day 5-7

5 μg/ 일, 정상적으로간에 2 mg, 기타조직에 2 mg 저장 - 흡수과정 : 위에서 R factor (haptocorrin) 와결합

30 (2) Vitamin B 12 - 유기금속화합물 (complex organometallic compound): corrin ring에 cobalt원자함유 - 최소영양요구량 : 2.5 μg/ 일, 정상적으로간에 2 mg, 기타조직에 2 mg 저장 - 흡수과정 : 위에서 R factor (haptocorrin) 와결합 십이지장에서 R factor와분리, 내인성인자 (intrinsic factor) 와결합 vitamin B 12 -IF 복합체가 ileal receptor에결합, 흡수 혈중에서 Vitamin B 12 - Transcobalamin II 복합체형성 간, 골수등의조직에서흡수

31 (3) 엽산 (folic acid, pteroylmonoglutamic acid) - 최소영양요구량 : 50 μg/ 일, 임신중요구량증대, 체내에정상적으로 5 20 mg 저장 (1/2은간 ) - 혈중엽산 : 주로 N 5 -methyltetrahydrofolate의형태로존재, 특이적수송체에의해세포내로이동 세포내에서 vitamin B 12 가관여하는 N 5 -methyl group 제거반응후, polyglutamate형태로변환 DNA 생성에관여 - Metabolic function : one-carbon moieties (methyl and formyl group) 전달반응 DNA 생합성에필수적 (4) 기타 - Vitamin B 6 (pyridoxine), Vitamin B 2 (riboflavin), niacin, Vitamin C (ascorbic acid), Vitamin E (tocopherol), Cobalt, Copper, Thyroxine, Amino acids Pteroic acid

3) 적혈구생성조절 - 신장내의산소분압 : erythropoiesis 의주요조절인자 Oxygen tension renal erythropoietic factor (released by the kidney) + plasma protein from the liver

32 3) 적혈구생성조절 - 신장내의산소분압 : erythropoiesis 의주요조절인자 Oxygen tension renal erythropoietic factor (released by the kidney) + plasma protein from the liver erythropoietin - Erythropoietin 분자량 34KD, 신장 (80 90%) 및간 (10 20%) 에서생성 산소분압이낮은환경에서생성, hemopoietic stem cell proerythroblast 과정촉진 - 만성신장질환 erythropoietin 결핍에의한빈혈

33 4. 적혈구내대사 에너지대사 - 혐기적해당과정 (anaerobic glycolysis)

34 5. 비정상적혈구 (abnormal red cells) - Anisocytosis ( 赤血球大小不同症 ): 크기변화 ; macrocytes, microcytes - Poikilocytosis ( 奇形赤血球症 ) : 형태변화 ; spherocytes, sickle cell * Red cell indicies ( 적혈구수치 ) : 적혈구이상을판정하는지표 ( 크기, 형태, 색깔 ) 1) 평균적혈구용적 (MCV, mean corpuscular volume index) : 정상성인 87±5 m 3 2) 평균적혈구혈색소 (MCH, mean corpuscular hemoglobin) : 정상성인 29±2 pg 3) 평균적혈구혈색소농도 (MCHC, mean corpuscular hemoglobin concentration) : 정상성인 34±2% 6. 적혈구의취약성 - tonicity 와 osmolarity - 고장액 (hypertonic solution) : 적혈구의 crenation - 저장액 (hypotonic solution) : 삼투성용혈 (osmotic hemolysis) - 삼투성취약성 (osmotic fragility) : 적혈구의용혈곡선 (fragility curve), 빈혈을유발하는각종질병상태에서곡선의좌우이동관찰

35 In hypertonic solution In hypotonic solution

7. 빈혈 (Anemia) - 빈혈은질환의증상 (sign of disease)

02, female 14.02); Hematocrit < 37% ( 정상치 : male 47.

05) - 증상 일반증상 : 호흡곤란 (shortness of breath), 빈맥

conjunctival mucosa ( 격막성점막의창백 ), 설사, 변비 특이증상 spoon nail

36 7. 빈혈 (Anemia) - 빈혈은질환의증상 (sign of disease) 이며혈액의산소운반능력저하에기인 - Hemoglobin < 12 g/dl ( 정상치 : male 16.02, female 14.02); Hematocrit < 37% ( 정상치 : male 47.05, female 42.05) - 증상 일반증상 : 호흡곤란 (shortness of breath), 빈맥 (tachycardia), 두통 (headache), 피로, 정신작업곤란, pallor of the conjunctival mucosa ( 격막성점막의창백 ), 설사, 변비 특이증상 spoon nail (koilonychia) : iron deficiency 황달 : 용혈성, 거대적혈구성 (megalobalstic) 빈혈 leg ulcer: sickle cell anemia bone deformity: thalassaemia major, 기타선천적용혈성빈혈

37 8. 적혈구과다증 (polycythemia) - 적혈구수 > 6 million/mm 3 ( 정상치 : 5x10 6 RBC/ mm 3 : 남 : x10 6 /mm 3 ; 여 : x10 6 /mm 3 ) 1) 생리적적혈구과다증 (physiologic polycythemia) (1) 상대적적혈구과다증 (relative-, stress- or pseudopolycythemia) - 혈중적혈구수의상대적증가 : 탈수, hemoconcentration ( 혈액농축 ) - 심혈관계질환 (e.g. myocardial ischemia), 이뇨제치료 (diuretic therapy), heavy smoking (2) secondary polycythemia (erythrocytosis); - 저산소증에의한 2차적인적혈구생성증가 : 만성적인저산소환경 ( 고산지대 ), 기타질환 (emphysema, pulmonary fibrosis, carbon monoxide poisoning) 2) Polycythemia vera ( 적혈구증가증 ) - a chronic, slowly progressive disease - 골수에서의혈구세포생성증가 (hyperplasia) - 원인 : 대개의경우잘모름 (idiopathic); 혈중산소부족과는관계없음, 골수, 신장, 뇌의종양? - 혈액점도증가, 심장및혈관에부담 (increased load), 혈전생성용이

38 C. 백혈구 - 7,000-10,000 leucocytes/ mm 3 1. 분류 1) 과립백혈구 (granulocytes ) - 다형핵백혈구 (polymorphonuclear leucocytes) : 호중구 (neutrophil), 호산구 (eosinophil), 호염기구 (basophil) 2) 무과립백혈구 (agranulocytes) - 단순한형태의핵 (simple, unlobulated nucleus, monomorphonuclear leucocyte) : 림프구 (lymphocyte), 단핵세포 (monocyte) 2. 기능 - 생체방어기능 : 해독작용 (detoxify poisons), 항체생성, 각종화학물질, 효소생성및분비 1) 식균작용 (phagocytosis) 2) 면역기능 (immunity)

40 백혈구의기능적성질 - 누출성 : 위족통과 (diapedisis) 에의해혈관통과 - 아메바성운동 (ameboid movement): 30~40 m/sec - 화학주성 (chemotaxis): 감염받은조직내에서생성된화학물질 (leucotaxin, necrosin, etc) 이백혈구를감염받은조직으로끌어당김 Diapedesis

41 3. 백혈구의특징 1) 호중구 (Neutrophil, 10-14μm) - 총백혈구의 % - 급성염증반응에관여 (act as phagocytes), myeloperoxidase 다량함유 2) 호산구 (Eosinophils, 10-12μm) - allergic disorder나기생충감염시증가 eosinophilia - 기능 : histamine 불활성화 항원-항체복합체탐식작용 fibrinolysin 분비 fibrin clot 용해 3) 호염기구 (Basophils, 8-12μm) - histamine 및 heparin 함유과립 allergy 반응및혈액응고방지계에중요한역할 4) 림프구 (Lymphocytes) - 체액성면역 (humoral immunity) 및세포성면역 (cell mediated immunity) 에중요한역할 - T-세포, B-세포 5) 단핵세포 (Monocytes, 16-20μm) - tissue macrophages: 급성염증반응, 면역반응에관여 * 망상내피조직계 (Reticuloendothelial system) : red bone marrow, 간, 비장, 림프절, loose connective tissue 등의일반명칭 : dying blood cell, platelets 파괴

42 4. 백혈구형성 (Leucopoiesis) - 과립구, 단구 (monocyte) : 골수에서발생, 성숙 - 림프구 : potential T-lymphocytes: 흉선, 비장에서성숙 ; potential B-lymphocytes; 소장의림프조직, 비장, 골수에서성숙 - Leucocytes-inducing factor (LIF) : granulopoiesis 촉진 - 백혈구의수명 : 호중구 : 수일 ; B-림프구, 몇몇 T-림프구 : 수년 5. 백혈구감소증 (Leucopenia) - 골수의결함 ( 세포발생, 성숙지체 ), 백혈구파괴등에기인 - 무과립세포증 (agranulocytosis) 호중구의뚜렷한감소가특징 골수억제에기인 (irradiation, cytotoxic drugs) 세균감염에대한저항성약화

43 6. 백혈구증가증 (Leucocytosis) - 비정상적인백혈구수의증가 : >500,000 cells/mm 3 -- leucocytosis 1) 생리적백혈구증가증 (physiological leucocytosis, 10,000-20,000/mm 3 ) - 신생아, 임신, 정서불안, 월경, 격심한운동 2) 병리적백혈구증가증 (pathological leucocytosis) - 세균, 바이러스감염, 대사및호르몬이상, allergies, 종양 3) 백혈병 (Leukemia) - 기능적으로결함이있는미성숙조혈세포의과도한증식 : malignant disease ( 악성질환 ) - 환경인자 : radiation, benzene, 유전적소인, RNA virus - 분류 : 세포또는조직의형태, 질환의이행과정및경과기간등에따라분류 (1) 급성백혈병 (acute leukemia, 87%) rapid onset, massive numbers of immature leucocytes, severe anemia 급성림프성백혈병 (acute lymphocytic leukemia, 30%) 급성골수성백혈병 (acute myelogenous leukemia, 70%) (2) 만성백혈병 (chronic leukemia, 13%) gradual onset and increased numbers of mature leucocytes, may lead to acute leukemia 빈혈, excessive bleeding(impairment of platelets), 국소또는전신감염

D. 혈소판 - 진정한세포가아님, 거대핵세포 (megakaryocyte) 유래 : 140,000-340,000/mm 3 6,000 thrombocytes/one megakaryocyte 1.

45 D. 혈소판 - 진정한세포가아님, 거대핵세포 (megakaryocyte) 유래 : 140, ,000/mm 3 6,000 thrombocytes/one megakaryocyte 1. 혈소판형성 (Thrombocytopoiesis) 1) 대부분의 megakaryocytes 는골수에위치 2) 일부 megakaryocyte 가타조직 ( 특히폐 ) 으로이동 혈소판형성 2. 형태적특성 1) 핵이없다 (small, membrane-bound bodies without nuclei) 2) granules, vesicles, microfilaments, microtubules, occasionally mitochondria 포함 3. 기능 1) 혈관손상시 : chemicals 유리 혈관수축 2) adhesive properties 3) 혈액응고인자생성 4. 기타 - 수명 : 약 1-2 주, 간, 비장의대식세포에의해파괴 - 비장 : 혈소판의주요저장장소, stress (hemorrhage, burns) 시 sympathetic stimulation of spleen 다량의혈소판을혈중으로방출 - Thrombocytopenia ( 혈소판감소증 ) : 혈중혈소판부족 ; 골수활성부전또는백혈병에기인 ; 과도한출혈 ( 예 ; skin, gastrointestinal tract)

46 Platelets form by budding off from megakaryocytes in the bone marrow Platelets: reddish or purple granules

48 Mesenchymal stem cells, or MSCs, are multipotent stromal cells that can differentiate into a variety of cell types, including: osteoblasts (bone cells), chondrocytes (cartilage cells),and adipocytes (fat cells)

49 III. 혈장 (Plasma) A. 혈장의성분 (composition of plasma) 성분분포 Water 90 % Protein 7 % Na +, K +, Cl -, HCO 3-, Ca ++ 1 % non-protein organic substances, fatty acids, glycerol 1 %

50 B. 단백성분 1. Albumin (MW 69,000) - 가장많은혈장단백 ( 약 40 g/l plasma), 간에서합성 - 교질삼투압형성 (colloid osmotic pressure) - 각종물질의운반체 : hormone (e.g. corticosteroids), bilirubin, fatty acids, 중금속, 약물 - ph 완충작용 (H2A HA - + H + ) 2. Globulin - 분자량이다양 - 지단백 (lipoprotein) : High-density lipoprotein, Low-density lipoprotein - 물질수송에관여 : thyroxine-binding globulin for thyroxine, transcortin for corticosteroid, transcobalamin for vitamin B 12, transferrin for iron, ceruloplasmin for copper, lipoproteins for lipid (LDL, HDL, VLDL) - 면역글로불린 (immunoglobulin); IgA, IgD, IgE, IgG, IgM - A/G 비율 (A/G ratio) : 정상치 ( 간장애 A/G 비율감소 ) 3. Fibrinogen - 간에서합성 - 혈액응고과정중불용성의 fibrin 으로변환 * 혈청 (serum) 과혈장 (plasma)

51 IV. 지혈 (Hemostasis) A. 지혈과정 혈관수축 (Vasoconstriction) 혈소판응집 (Platelet aggregation) 혈전형성 (Formation of blood clot) 혈전수축및용해 (Clot retraction and dissolution) 1. Vascular phase - Vascular spasm: 자율신경계 (autonomic nervous system) 및혈관수축물질 (serotonin등) 작용 2. Platelet phase : 혈소판응집에의한일시적혈소판마개형성 (platelet plug) 1) Step 1: 혈소판의혈관내부착 2) Step 2: 혈소판마개 (platelet plug) 형성 primary aggregation, secondary aggregation, release reaction 3. Coagulation phase : fibrin에의한 plug의안정화 1) Stage I: prothrombin converting factor (plasma thromboplastin) 의형성 내인성경로 (intrinsic pathway) : 모든인자가혈액내존재 외인성경로 (extrinsic pathway) : tissue thromboplastin (factor III) 가손상된조직및세포에서분비 2) Stage II: prothrombin의 thrombin으로의변환 3) Stage III: fibrinogen의 fibrin으로의변환 4. Clot retraction and dissolution (fibrinolysis, 纖維素溶解 ) Clot retraction: thrombosthenin (platelet factor) 섬유소용해 (fibrinolysis): plasmin

53 혈소판응축 (aggregation) 은혈전 (blood clot) 을형성 혈소판마개 (Platelet plug) ADP: Adenosine diphosphate TxA 2 : Thromboxane A 2

54 주요혈액응고관련인자

55 혈전 (Blood clot) RBC Platelet Fibrin thread XIII (Fibrin stablizing factor)

56 응고반응 손상된조직에서시작 콜라겐단백질에부착되어있는응고인자들의활성화로시작 X: Stuart-Power factor (serine protease) Cross-linked fibrin

57 Vitamin K (Factors II, VII, IX, X)

58 Vitamin K 2-methyl-1,4-naphtoquinone skeleton Phylloquinone Menaquinone Menadione Vit K Antagonists

Nicholas II of Russia and his family, photographed c.

59 B. 혈액응고이상 (Clotting disorder) 1) 혈관이상 (vascular defects) 2) 혈소판부족 (thrombocytopenia) 3) 혈소판기능부전 4) 응고인자부전 5) Inherited clotting disorder : Hemophilia ( 혈우병 ) Hemophilia A ( 응고인자 VIII 결함 ) Hemophilia B ( 응고인자 IX 결함 ) Czar Nicholas II of Russia and his family, photographed c. 1916, showing his wife Alexandra (who was a carrier of hemophilia), his four daughters, and (in the foreground) his son Alexis, perhaps the most famous European royal with hemophilia.

60 V. 혈액형 (Blood Type) A. ABO 식혈액형 Landsteiner ( ) 응집원 (agglutinogen), 응집소 (agglutinin) Blood Types AA or AO = Type A BB or BO = Type B OO = Type O AB = Type AB GalNAc: N-acetylgalactosamine ABO 혈액형의빈도수 Phenotype Genotype Antigens Agglutinin Frequency (%) Korean American O OO O Anti-A, Anti-B A AA or AO A Anti-B B BB or BO B Anti-A AB AB AB None

61 ABO 식혈액형 Universal Donor Universal Recipient

62 ABO 혈액형항체 (agglutinin) 의기원 : - 출생직후혈중에는 A 와 B 항체가존재하지않음 6 개월이내에생성 유전적으로결정?

63 B. Rh 혈액형 - 수혈시가장중요 (Landsteiner 1941) - 붉은털원숭이 (rhesus monkey) 적혈구의항체에대한반응성 1. Rh 인자 (Rh factor) - anti-rhesus antibodies에의해인식여부 : Rh-positive, Rh-negative - 주요 6 항원 (C,D,E,c,d,e) 한쌍으로유전 : 예 ) CDE/cDE D-antigen에따라구분 : Rh-positive (genotype DD or Dd) Blood type European Asian Black-American African Rh + 85% Rh - 15% 95% <1% 95% 5% 100% 2. 항체의생성 - 수혈및임신 ( 임신시 : 항체에의한태아적혈구파괴 erythroblastosis fetalis) 3. Erythroblastosis fetalis ( 태아적모구증, 胎兒赤母球症 ) first child; generally no harm second child : 3% sign of erythroblastosis fetalis third child : 10% sign of erythroblastosis fetalis * 핵황달 ( 核黃疸, Kernicterus) * erythrobalstosis fetalis의예방과치료 : 출산직후산모에 anti-d antibody [anti-d prophylaxis (RhoGAM)] 처리

64 Rh - mother w/rh - baby- no problem Rh + mother w/rh - baby no problem Rh - mother w/rh + baby problem

65 C. 교차시험 (cross matching) - 주교차시험 (major cross matching) : 공혈자의적혈구 + 수혈자의혈청 - 부교차시험 (minor cross matching) : 수혈자의적혈구 + 공혈자의혈청 D. 혈액형의생물학적의의 1) 면역반응 (Immunoreactions) 2) 불임및유산 (Infertility and early fetal loss) 3) 기타질환과의관련성 - Type O "nonsecretors" : type A 또는 B secretors에비해십이지장궤양발현율이 2배 - Type A : type O에비해 salivary glands, stomach, pancreas 발암율이높다 - Rh null type (Rh antigen이결핍 ): 용혈정도가높다.

RBC (Red Blood Cell) 1. major function 1 gas 운반 : transport of oxygen - RBC내의 hemoglobin에산소가결합되어 lung에서 tissue로운반 : transport of carbon dioxide - RBC내

RBC (Red Blood Cell) 1. major function 1 gas 운반 : transport of oxygen - RBC내의 hemoglobin에산소가결합되어 lung에서 tissue로운반 : transport of carbon dioxide - RBC내 Blood Physiology Blood 의구성 1) cellular component (45%) (1) RBC (erythrocyte) (2) WBC (leukocyte) 1 granulocyte : neutrophil eosinophil basophil 2 lymphocyte 3 monocyte (3) platelet (thrombocyte) 2) plasma