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Introduction   介紹

What is hematology?  什麼是血液學?

Hematology  血液學

  • A branch of medical specialty
    醫學專業的一個分支
  • Hematologist as specialist
    血液學家作為專家
  • Clinical hematology  臨床血液學
  • Laboratory hematology  實驗室血液學
  • Research  研究

Blood:  血:

Composition, physiology, hematopoiesis, hemostasis
成分、生理學、造血、止血

Blood and Circulation  血液和迴圈

  • Blood makes up about 7 % 7 % 7%7 \% of the weight of a human body, with a volume of about 5 liters in an average adult.
    血液大約 7 % 7 % 7%7 \% 占人體的重量,普通成年人的體積約為 5 升。
  • It is made of fluid (plasma) and cellular elements (RBC, WBC, and platelets)
    它由液體(血漿)和細胞成分(RBC、WBC 和血小板)組成
  • It is slightly alkaline, with a pH of 7.4
    呈微鹼性,pH 值為 7.4

  • Its color varies from bright to dark red
    它的顏色從亮紅色到深紅色不等
  • It has a salty metallic taste
    它有鹹的金屬味

Blood  

Blood along with the heart and the blood vessels (e.g.veins and arteries)comprises the circulatory system of the body.
血液與心臟和血管(例如靜脈和動脈)一起構成了身體的循環系統。
The circulatory system helps in maintaining balanced conditions within the body(i.e. homeostasis體內動觬平衡).
循環系統有助於維持體內的平衡狀態(即體內平衡)。

Blood:Functions  血液:功能

-Transports oxygen and nutrients
- 輸送氧氣和營養物質

-Removes CO 2 CO 2 CO_(2)\mathrm{CO}_{2} and other by-products of cell activity
- 去除 CO 2 CO 2 CO_(2)\mathrm{CO}_{2} 細胞活性的其他副產品

-Maintains homeostasis體内動態平衡,growth and tissue repair
-維持體內平衡、生長和組織修復

-Participates in the defense against infection
- 參與防禦感染

-Participates in hemostasis止血機制
-參與止血制

-Participates in body heat distribution and regulation
- 參與體熱分配和調節

Blood - Plasma  血液 - 血漿

Straw colored fluid made of water ( 90 % 90 % ∼90%\sim 90 \% ), other contents include:
稻草色水製成的液體 ( 90 % 90 % ∼90%\sim 90 \% ),其他內容物包括:

Proteins make the bulk of the solutes:
蛋白質構成了大部分溶質:

Albumins (60%), manufactured in the liver are the most abundant, for oncotic(osmotic) pressure
肝臟中製造的白蛋白 (60%) 最豐富,用於滲透(滲透)壓

Globulins (36%) are immune bodies Fibrinogen (4%) for blood clotting Clotting factors
球蛋白 (36%) 是凝血因數的免疫體纖維蛋白原 (4%)

Nutrients: glucose, amino acids, lipoproteins, cholesterol
營養物質:葡萄糖、氨基酸、脂蛋白、膽固醇

Electrolytes: Na + , K + , Ca + + , Mg + + , H + , Cl , HCO 3 , PO 4 , SO 4 Na + , K + , Ca + + , Mg + + , H + , Cl , HCO 3 , PO 4 , SO 4 Na^(+),K^(+),Ca^(++),Mg^(++),H^(+),Cl^(-),HCO_(3)^(-),PO_(4)^(--),SO_(4)^(--)\mathrm{Na}^{+}, \mathrm{K}^{+}, \mathrm{Ca}^{++}, \mathrm{Mg}^{++}, \mathrm{H}^{+}, \mathrm{Cl}^{-}, \mathrm{HCO}_{3}^{-}, \mathrm{PO}_{4}^{--}, \mathrm{SO}_{4}^{--} Waste: urea, creatinine, uric acid,
電解質: Na + , K + , Ca + + , Mg + + , H + , Cl , HCO 3 , PO 4 , SO 4 Na + , K + , Ca + + , Mg + + , H + , Cl , HCO 3 , PO 4 , SO 4 Na^(+),K^(+),Ca^(++),Mg^(++),H^(+),Cl^(-),HCO_(3)^(-),PO_(4)^(--),SO_(4)^(--)\mathrm{Na}^{+}, \mathrm{K}^{+}, \mathrm{Ca}^{++}, \mathrm{Mg}^{++}, \mathrm{H}^{+}, \mathrm{Cl}^{-}, \mathrm{HCO}_{3}^{-}, \mathrm{PO}_{4}^{--}, \mathrm{SO}_{4}^{--} 廢物:尿素、肌酐、尿酸、

bilirubin Gases: O 2 , CO 2 , N 2 O 2 , CO 2 , N 2 O_(2),CO_(2),N_(2)\mathrm{O}_{2}, \mathrm{CO}_{2}, \mathrm{~N}_{2}
膽紅素氣體: O 2 , CO 2 , N 2 O 2 , CO 2 , N 2 O_(2),CO_(2),N_(2)\mathrm{O}_{2}, \mathrm{CO}_{2}, \mathrm{~N}_{2}

Protein bound hormones  蛋白結合激素
Plasma without clotting factors is called “serum”
不含凝血因數的血漿稱為“血清”

Plasma= Blood fluid with no cells
血漿 = 無細胞的血液

(Serum= Blood fluid with no cells and no clotting factors)
(血清 = 無細胞且無凝血因數的血液)
Plasma: 55% of blood volume
血漿:血容量的55%
Blood Cells: 45% of blood volume, with RBC as the greatest contributor
血細胞:佔血容量的45%,其中紅細胞是最大的貢獻者

  • ESR (Erythrocyte Sedimentation Rate) rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour.
    ESR(紅細胞沉降率):抗凝全血中的紅細胞在標準化管中一小時內下降的速率。
  • Hematocrit vol% of RBCs in blood
    血細胞比容體積 血液中紅細胞的百分比
Female: 36.1-44.3%  女性:36.1-44.3%
Non specific, Increased in pregnancy, inflammation, multiple myeloma
非特異性、妊娠期升高、炎症、多發性骨髓瘤

Blood - Cell Types & Function
血液 - 細胞類型和功能

Red blood cells (RBC) carry oxygen
紅細胞 (RBC) 攜帶氧氣
White blood cells (WBC) fight infection
白細胞 (WBC) 抵抗感染
Platelets  血小板
clotting  凝血

Cells in the blood  血液中的細胞

  • RBC (size: 6-8 microns) life span: ~100-120 days. (can be stored in the Blood Bank for 42 days.)
    紅細胞(大小:6-8 微米)壽命:~100-120 天。(可在血庫中儲存 42 天。
  • WBC (size: 10 to 17 microns) life span: 1-9 days. (must be used within 24 hours)
    WBC(尺寸:10 至 17 微米)壽命:1-9 天。(必須在 24 小時內使用)
  • Platelets (2-4 microns) life span: 8-10 days. (can be stored in the Blood Bank for 5 days.)
    血小板(2-4 微米)壽命:8-10 天。(可在血庫中保存 5 天。

Formation of Blood cells Hemopoiesis
血細胞的形成 造血

Hormones:
RBC:
激素: 紅細胞:

Erythropoietin Platelets:
促紅細胞生成素血小板:
Thrombopoietin WBC’s:  血小板生成素 WBC:
Colony-  殖民地-
Stimulating  刺激
Factors  因素

WBC Anatomy and Function Neutrophil
WBC 解剖和功能 中性粒細胞

  • Make up 60 to 70% of WBC’s
    占 WBC 的 60% 至 70%
  • lobulated nucleus, 2-5 lobes (increase with cell age)
    分葉狀核,2-5 個裂片(隨著細胞年齡的增加而增加)
  • Phagocytic cells, engulf bacteria
    吞噬細胞,吞噬細菌
  • Increase: stress, burns, bacterial infections
    增加:壓力、燒傷、細菌感染
  • Decrease: Radiation exposure, B12/folate deficiency
    減少:輻射暴露、B12/葉酸缺乏

WBC Anatomy and Function Eosinophil
WBC 解剖和功能 嗜酸性粒細胞

  • Make up 2-4% of WBC’s
    占 WBC 的 2-4%
  • lobulated nucleus, 2-3 lobed
    分葉核,2-3 裂片
  • Combat histamines in allergic reactions
    對抗過敏反應中的組胺
  • Phagocytic on antigen/antibody complexes
    抗原/抗體複合物上的吞噬作用
  • Destroy certain parasitic worms
    消滅某些寄生蠕蟲
  • Increase: allergic reactions, parasitic infections, autoimmune disease
    增加:過敏反應、寄生蟲感染、自身免疫性疾病

WBC Anatomy and Function Monocytes
WBC 解剖和功能單核細胞

  • Make up 3-8 % of WBC’s
    占 WBC 的 3-8%
  • lobulated nucleus, kidney-shaped
    分葉核,腎形
  • Phagocytic cells  吞噬細胞
  • Increase: Viral and fungal infections, tuberculosis, and some leukemias
    增加:病毒和真菌感染、結核病和一些白血病
  • Decrease: bone marrow depression treatment with cortisol
    降低:皮質醇治療骨髓抑制

WBC Anatomy and Function Basophil
WBC 解剖和功能 嗜鹼性粒細胞

  • Make up 0.5 to 1 % 1 % 1%1 \% of WBC’s
    占 WBC 的 0.5 到 1 % 1 % 1%1 \%
  • Cytoplasm filled with large deep blue-purple granules
    細胞質充滿大的深藍紫色顆粒
  • Liberate heparin and histamines during allergic reactions
    在過敏反應期間釋放肝素和組胺
  • Intensify inflammatory response
    加強炎症反應
  • Increase: Allergic reactions, leukemia, cancers, hypothyroidism
    增加:過敏反應、白血病、癌症、甲狀腺功能減退症
  • Decrease: Pregnancy, ovulation, stress, hyperthyroidism
    減少:懷孕、排卵、壓力、甲狀腺功能亢進

WBC Anatomy and Function Lymphocytes
WBC 解剖和功能淋巴細胞

  • Make up 20 to 25% WBC’s
    佔 20% 至 25% 的白細胞
  • Nucleus is round or slightly indented
    細胞核呈圓形或略微凹陷
  • adaptive/aquired Immunity
    適應性/獲得性免疫
  • B cells produce antibodies
    B 細胞產生抗體
  • T cells attack viruses, cancer cells, and transplanted tissues
    T 細胞攻擊病毒、癌細胞和移植組織
  • Natural killer cells attack infectious microbes and tumor cells
    自然殺傷細胞攻擊感染性微生物和腫瘤細胞

Red blood cells  紅細胞

  1. biconcave shape  雙凹形

2) no nucleus  2) 無核

rarr\rightarrow extra space inside
rarr\rightarrow 內部空間

3) contain hemoglobin  3) 含有血紅蛋白
rarr\rightarrow the oxygen carrying molecule
rarr\rightarrow 攜氧分子

250 250 rarr250\rightarrow 250 million molecules
250 250 rarr250\rightarrow 250 萬分子

increases the  增加
surface area so more / cell oxygen can be carried
表面積因此可以攜帶更多/細胞氧氣
Erythrocyte Sedimentation rate (ESR)= 0 to 20 mm / hr mm / hr mm//hr\mathrm{mm} / \mathrm{hr}
紅細胞沉降率 (ESR) = 0 至 20 mm / hr mm / hr mm//hr\mathrm{mm} / \mathrm{hr}

Platelet  血小板

  • Blood clotting  血液凝固

Circulatory System  循環系統

  • Intravascular Compartment
    血管內隔室
  • Extravascular Compartment
    血管外隔室
Extravasation of blood cells
血細胞外滲
  • Diapedesis  尿道
  • Hemorrhage  出血

Blood Smear  血塗片

RBC  紅細胞
WBC  白細胞
Giemsa stain  Giemsa 染色
Platelets  血小板

Hematopoiesis  造血

Sites of blood production
造血部位

  • In fetus: liver and spleen
    胎兒:肝臟和脾臟
  • In adults: bone marrow (medulla) vertebrae, pelvis, sternum, ribs, clavicles, skull, long bones of arms and legs
    成人:骨髓(延髓)椎骨、骨盆、胸骨、肋骨、鎖骨、顱骨、手臂和腿部的長骨
  • What is extramedullary hematopoiesis?
    什麼是髓外造血術?
  • production of blood cells outside the bone marrow, when there is inadequate blood cell production in bone marrow. Mainly liver and spleen. Others like lungs, kidneys, peritoneal cavity.
    當骨髓中血細胞生成不足時,骨髓外產生血細胞。主要是肝臟和脾臟。其他如肺、腎、腹膜腔。


    marrow www.fotosearch.com
    骨髓 www.fotosearch.com

Bone Marrow Produces  骨髓產生



Figure 4-7. Human blood cells form a smear after Wright’s stain. A and D, Neutrophilic leukocytes. B and E, Eosinophilic leukocytes. C. Basophilic leukocyte. F. Plasma cell; this is not a normal constituent of the peripheral blood but is included here for comparison with the nongranular leukocytes. G and H H HH, Small lymphocytes. t t tt, Medium lymphocytes. J J JJ, K K KK, and L, Monocytes.
圖 4-7.人類血細胞在 Wright 染色后形成彌散片。A 和 D,中性粒細胞白細胞。B 和 E,嗜酸性粒細胞白細胞。C. 嗜鹼性白細胞。F. 漿細胞;這不是外周血的正常成分,但此處包括用於與非顆粒白細胞進行比較。G 和 H H HH ,小淋巴細胞。 t t tt ,中等淋巴細胞。 J J JJ K K KK 和 L,單核細胞。
Reticuloyte Count 1% of RBCs
網狀細胞計數 1% 的紅細胞

Hemostasis  止血

Hemostasis  止血

  • capability of activation of clot formation and clot lysis in order to prevent hemorrhage without causing thrombosis
    啟動凝塊形成和凝塊溶解的能力,以防止出血而不引起血栓形成
  • Primary hemostasis: platelet aggregation rarr\rightarrow for plug
    原發性止血:血小板聚集 rarr\rightarrow 導致栓塞
  • Secondary hemostasis: involve clotting cascade to form fibrin. Fibrin then form mesh with trap blood cells to form clot, which dried to form a scab.
    繼發性止血:涉及凝血級聯反應形成纖維蛋白。然後纖維蛋白與捕獲血細胞形成網狀物形成凝塊,凝塊乾燥形成結痂。
  • Fibrinolysis: enzymatic breakdown of fibrin in blood clot
    纖維蛋白溶解:血凝塊中纖維蛋白的酶促分解

Steps in blood clotting  血液凝固的步驟

Vascular spasm  血管痙攣

Collagen fibers  膠原纖維

Fibrin  纖維素

Platelet plug formation  血小板栓形成

HEMOSTASI  止血

DEPENDENT UPON:  依賴:

Vessel Wall Integrity  容器壁完整性
Adequate Numbers and  足夠的人數和
Proper Functioning of Platelets
血小板的正常功能

Adequate Levels of Clotting Factors
足夠的凝血因數水準

and Proper Function of Fibrinolytic
和纖溶劑的正常功能

Pathway  

Vessel Injury  血管損傷

Endothelial Damage  內皮損傷
rarr\rightarrow disrupts the smooth lining of the endothelial cells.
rarr\rightarrow 破壞內皮細胞的光滑襯裡。

rarr\rightarrow Exposure of Collagen:
rarr\rightarrow 膠原蛋白的暴露:

rarr\rightarrow triggers platelets adhesion, initiates subsequent clotting steps.
rarr\rightarrow 觸發血小板粘附,啟動後續的凝血步驟。

rarr\rightarrow Damaged endothelial cells release various factors that promote clotting.
rarr\rightarrow 受損的內皮細胞會釋放各種促進凝血的因數。

Coagulation Cascade:  凝血級聯反應:

Intrinsic Pathway:  內在途徑:

Activation of Factor XII by contacts with collagen or damaged cells
通過與膠原蛋白或受損細胞接觸啟動因數 XII

rarr\rightarrow triggers a series of enzymatic reactions, resulted in activation of factor X .
rarr\rightarrow 觸發一系列酶促反應,導致因數 X 的啟動。

rarr\rightarrow enters the common pathway.
rarr\rightarrow 進入 Common Pathway。

Extrinsic Pathway:  外源性途徑:

Activation of factor VII by Tissue Factors
組織因數啟動因數 VII

rarr\rightarrow triggers a series of enzymatic reactions, resulted in activation of factor X X XX
rarr\rightarrow 觸發一系列酶促反應,導致因數 X X XX 啟動

rarr\rightarrow enter the common pathway
rarr\rightarrow 進入 Common Pathway

Common Pathway:  常見途徑:

Prothrombin is converted to thrombin by activated factor X X XX, in collaboration with activated factor V . Thrombin enzyme converts fibrinogen to fibrin.
凝血酶原通過活化因數 X X XX 轉化為凝血酶 ,與活化因數 V 合作。凝血酶將纖維蛋白原轉化為纖維蛋白。

Fibrin Clot Formation:  纖維蛋白凝塊形成:

Positive Feedback Loop: Thrombin positively feedback on the clotting cascade and amplify the cascade. A robust clot formation resulted.
正反饋迴路:凝血酶對凝血級聯反應產生正反饋並放大級聯反應。導致形成強大的凝塊。
Fibrin form a fibrous mesh that traps platelets and red blood cells, solidifying the clot.
纖維蛋白形成纖維網,捕獲血小板和紅細胞,使凝塊凝固。
Platelet Activation and Aggregation: Thrombin also activates platelets, causing them to release clotting factors, change shape, and clump together, further strengthening the clot.
血小板活化和聚集:凝血酶還激活血小板,使它們釋放凝血因數、改變形狀並聚集在一起,進一步加強凝塊。

Anticoagulation, Fibrinolysis:
抗凝、纖維蛋白溶解:

  1. Antithrombin Regulation: Antithrombin, a glycoprotein, inactivates thrombin and other clotting factors to prevent excessive clot formation and unwanted spread.
    抗凝血酶調節:抗凝血酶是一種糖蛋白,可滅活凝血酶和其他凝血因數,以防止過度凝塊形成和不必要的擴散。
  2. Protein C C CC and S S SS Activation: Thrombin activates protein C, which, in collaboration with protein S, degrades activated clotting factors, further limiting clot growth.
    蛋白質 C C CC S S SS 活化:凝血酶啟動蛋白 C,蛋白 C 與蛋白 S 協同降解活化的凝血因數,進一步限制凝塊生長。
  3. Plasminogen Conversion: As the wound heals, the inactive plasminogen is
    纖溶酶原轉化:隨著傷口癒合,無活性纖溶酶原

    converted into the active enzyme plasmin. Plasmin degrades fibrin clots.
    轉化為活性酶纖溶酶。纖溶酶降解纖維蛋白凝塊。