Anemias幻灯片.ppt
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参见附件(573KB)。
Anemias
CUF-S (造血干C、红系祖C)
↓
proerythroblast (原始RBC)
↓
Basophilicnormoblast ( 早幼RBC)
↓
Polychromaticmormoblast(中幼RBC)
↓
Orthrochromaticnormoblast (晚幼RBC)此阶段脱核进入血循环
↓
Reticulocyte (网织RBC)
经特殊染色见到丝状或网状结构, 保留残余线粒体和核糖，仍有继续合成Hb的能力(从早幼RBC开始)
↓
erythrocyte
Definition
? A reduction below normal in the concentration of hemoglobin , the mass of red blood cells and/or the hematocrit in the blood.
Factors influencing Hb concentration
Sex
Age
Altitude
Alterations in plasma volume
★Classification
Morphologic classification
Kinetic classification
(Classified according to etiology and pathogenesis)
一、Decreased erythropoiesis
? Proliferation and differentiation
abnormalities of SC
HSC: AA, Fanconis anemia, MDS
Erythrocytic progenitors : Pure red cell aplasia, Anemias caused by kidney failure andendocrine disorders
? Marrow infiltration
leukemias
carcinoma
multiple myeloma
myelofibrosis
Malignant histocytosis
? Production and maturation blockages of differentiating cells
DNA synthesis blockage : Vit B12, falic acid difficiencies, 嘌呤和嘧啶 metabolic defects→Megaloblastic anemia
Hb synthesis blockage :
Hemeproduction defect : IDA
Heptoglobin production defect :Thalassemias
4Unknown reason or several mechanisms
Sideroblastic anemia
Chronic disease anemias (chronic inflummation, infections, 尿毒症, Hepatic disorders, neoplasm, connective tissue disease, endocrine disorders)
二、Accelerated destruction of red cells
1)Endogenous (intra-erythrocyte defects) 1.Membrane defects of erythrocytes :
Hereditary :Hereditary spherocytosis (HS)
Hereditasy elliplocytosis (HE)Acquired : PNH
2.Enzymedefects
Glucose--6--Phosphate Dehydrogenase(G6PD) deficiency
Pyruvate kinase deficiency
3.Abnormal haptogllbin synthesis :
Sickle cell anemia, Hemoglobinopathies
Thalassemias
2)Extragenous
? Immune HA : AIHA, neonatal HA,mismatched transfusion, drug-induced HA
2. Mechanical : march hemoglobinuria , cardiac valves prostheses, microangiopathic hemolytic disorders.
3. Due to chemical, physical or microrganisms :
Chemical toxin-and drug-induced hemolysis, large scale burned patient (severe burned patient)
Infection-induced , benzene, radiation
microrganisms: Malaria, virus, etc.
4. Increased damage of monocyte-macrocyte system : Hypersplenism
三.Blood loss
◆Acute posthemorrage anemia
◆Chronic bleeding →IDA
Classified accordind to the proliferative situations of BM
Hyperplastic anemias (增生性贫血):
Hemolytic anemia
Anemia caused by blood loss
Megaloblastic anemia, IDA
Hypoplastic anemias (增生减低性贫血): AA
Clinicalmanifestations
Factors influencing symptoms:
Severity of anemia Hb<80~90g/L→symptoms
Rapidity of anemia
Abrupt loss of 20% of the circulating blood volume → marked pallor, postural hypotension and tachycardia (心动过速)
The loss of 50% → severe shock even dealth.
In contrast, the gradual loss (even Hb 30~40g/L) of the circulating red cell mass in a patient with pernicious anemia may occur without any symptoms at all.
Why?
? Red cell 2, 3 DPG
(RBC内2, 3-二磷酸甘油酸)↑
In anemia the oxyhemoglobin dissociation curve usually shifts (右移) in a manner to increase the quantity of oxygen released in tissues without oppeciably altering the quantity of osygen bound in the lungs. Red cell 2,3 diphophoglycerate (2,3 DPG) regularly increases in anemic patients to mediate this effect. Maximum elevation of RBC 2,3 DPG increases oxygen delivery only about 30 per cent , but this is a highly efficient form ofcompensation requiring no significant expenditure of energy.
The aged patients or patients with vascular or cardiac diseases may not stand to milder anemia.
The level of anemia at which symptoms occur is highly variable among individuals as would be expected from the widely differing degrees of physical activity , physical conditioning , circulatory adequacy , and sensitivity or stoicism of the population.
1.General manifestations:
(Nonspecific and reflect tissue hypoxia)
Tiredness, fatigue→ the most common, the earliest symptom
Pallor of skin and mucosa→sharedchracteristic
2.Cardiovascular systems :
Palpitations and dyspnea on exertion,breathlessness→common symptoms
Valvulae murmurs
Hb <30g/L, >3m→Anemic cardic disease
3. Neurologic system :
Headache, Dizziness,Confusion, decreased mental acuity (记忆力衰退),晕厥，注意力不集中，失眠，耳鸣.
severe anemia→Coma
4 .Digestive system:
Anorexia (食欲不振)，厌食→early megaloblastic anemia
Nausea(恶心)，flatulence(胀气)，diarrhoea(腹泻) or constipation (便秘)
lingual abnormalities (舌的改变)：common
5.Genitourinary system:
severe anemia→ polyurea (多尿),hypobaric urine (尿比重低),proteinuria (蛋白尿): 肾小球滤过功能和小管分泌及回收功能障碍
female:disturbed menstruation (月经紊乱), 性功能减退多见
6.Other:
7.Manifestations of underlying disease:
The process of correct diagnosis is the one of differential diagnosis.
Steps: 1. To Establish the type of anemia
2. To find out the cause or underlying diseases of anemia
Diagnosis
? Cause or inducer of anemia:
Nutrition, special habits for food change in stool habits: stool Guaiacs in all
profession
influation of surrounding environment chronic diseases
menstruation, marriage
一.History
2. Developingprocesses, severity & complications of anemias
Duration and onset of symptoms
3.Important laboratory results, diagnosis, treatments and their effects.
skin and mucosa: pallor, jaundice,petechiae
hair and nails
adenopathy (淋巴结肿大) hepatomegaly-splenomegaly
neurologic abnormalities......（后略) ......
Anemias
CUF-S (造血干C、红系祖C)
↓
proerythroblast (原始RBC)
↓
Basophilicnormoblast ( 早幼RBC)
↓
Polychromaticmormoblast(中幼RBC)
↓
Orthrochromaticnormoblast (晚幼RBC)此阶段脱核进入血循环
↓
Reticulocyte (网织RBC)
经特殊染色见到丝状或网状结构, 保留残余线粒体和核糖，仍有继续合成Hb的能力(从早幼RBC开始)
↓
erythrocyte
Definition
? A reduction below normal in the concentration of hemoglobin , the mass of red blood cells and/or the hematocrit in the blood.
Factors influencing Hb concentration
Sex
Age
Altitude
Alterations in plasma volume
★Classification
Morphologic classification
Kinetic classification
(Classified according to etiology and pathogenesis)
一、Decreased erythropoiesis
? Proliferation and differentiation
abnormalities of SC
HSC: AA, Fanconis anemia, MDS
Erythrocytic progenitors : Pure red cell aplasia, Anemias caused by kidney failure andendocrine disorders
? Marrow infiltration
leukemias
carcinoma
multiple myeloma
myelofibrosis
Malignant histocytosis
? Production and maturation blockages of differentiating cells
DNA synthesis blockage : Vit B12, falic acid difficiencies, 嘌呤和嘧啶 metabolic defects→Megaloblastic anemia
Hb synthesis blockage :
Hemeproduction defect : IDA
Heptoglobin production defect :Thalassemias
4Unknown reason or several mechanisms
Sideroblastic anemia
Chronic disease anemias (chronic inflummation, infections, 尿毒症, Hepatic disorders, neoplasm, connective tissue disease, endocrine disorders)
二、Accelerated destruction of red cells
1)Endogenous (intra-erythrocyte defects) 1.Membrane defects of erythrocytes :
Hereditary :Hereditary spherocytosis (HS)
Hereditasy elliplocytosis (HE)Acquired : PNH
2.Enzymedefects
Glucose--6--Phosphate Dehydrogenase(G6PD) deficiency
Pyruvate kinase deficiency
3.Abnormal haptogllbin synthesis :
Sickle cell anemia, Hemoglobinopathies
Thalassemias
2)Extragenous
? Immune HA : AIHA, neonatal HA,mismatched transfusion, drug-induced HA
2. Mechanical : march hemoglobinuria , cardiac valves prostheses, microangiopathic hemolytic disorders.
3. Due to chemical, physical or microrganisms :
Chemical toxin-and drug-induced hemolysis, large scale burned patient (severe burned patient)
Infection-induced , benzene, radiation
microrganisms: Malaria, virus, etc.
4. Increased damage of monocyte-macrocyte system : Hypersplenism
三.Blood loss
◆Acute posthemorrage anemia
◆Chronic bleeding →IDA
Classified accordind to the proliferative situations of BM
Hyperplastic anemias (增生性贫血):
Hemolytic anemia
Anemia caused by blood loss
Megaloblastic anemia, IDA
Hypoplastic anemias (增生减低性贫血): AA
Clinicalmanifestations
Factors influencing symptoms:
Severity of anemia Hb<80~90g/L→symptoms
Rapidity of anemia
Abrupt loss of 20% of the circulating blood volume → marked pallor, postural hypotension and tachycardia (心动过速)
The loss of 50% → severe shock even dealth.
In contrast, the gradual loss (even Hb 30~40g/L) of the circulating red cell mass in a patient with pernicious anemia may occur without any symptoms at all.
Why?
? Red cell 2, 3 DPG
(RBC内2, 3-二磷酸甘油酸)↑
In anemia the oxyhemoglobin dissociation curve usually shifts (右移) in a manner to increase the quantity of oxygen released in tissues without oppeciably altering the quantity of osygen bound in the lungs. Red cell 2,3 diphophoglycerate (2,3 DPG) regularly increases in anemic patients to mediate this effect. Maximum elevation of RBC 2,3 DPG increases oxygen delivery only about 30 per cent , but this is a highly efficient form ofcompensation requiring no significant expenditure of energy.
The aged patients or patients with vascular or cardiac diseases may not stand to milder anemia.
The level of anemia at which symptoms occur is highly variable among individuals as would be expected from the widely differing degrees of physical activity , physical conditioning , circulatory adequacy , and sensitivity or stoicism of the population.
1.General manifestations:
(Nonspecific and reflect tissue hypoxia)
Tiredness, fatigue→ the most common, the earliest symptom
Pallor of skin and mucosa→sharedchracteristic
2.Cardiovascular systems :
Palpitations and dyspnea on exertion,breathlessness→common symptoms
Valvulae murmurs
Hb <30g/L, >3m→Anemic cardic disease
3. Neurologic system :
Headache, Dizziness,Confusion, decreased mental acuity (记忆力衰退),晕厥，注意力不集中，失眠，耳鸣.
severe anemia→Coma
4 .Digestive system:
Anorexia (食欲不振)，厌食→early megaloblastic anemia
Nausea(恶心)，flatulence(胀气)，diarrhoea(腹泻) or constipation (便秘)
lingual abnormalities (舌的改变)：common
5.Genitourinary system:
severe anemia→ polyurea (多尿),hypobaric urine (尿比重低),proteinuria (蛋白尿): 肾小球滤过功能和小管分泌及回收功能障碍
female:disturbed menstruation (月经紊乱), 性功能减退多见
6.Other:
7.Manifestations of underlying disease:
The process of correct diagnosis is the one of differential diagnosis.
Steps: 1. To Establish the type of anemia
2. To find out the cause or underlying diseases of anemia
Diagnosis
? Cause or inducer of anemia:
Nutrition, special habits for food change in stool habits: stool Guaiacs in all
profession
influation of surrounding environment chronic diseases
menstruation, marriage
一.History
2. Developingprocesses, severity & complications of anemias
Duration and onset of symptoms
3.Important laboratory results, diagnosis, treatments and their effects.
skin and mucosa: pallor, jaundice,petechiae
hair and nails
adenopathy (淋巴结肿大) hepatomegaly-splenomegaly
neurologic abnormalities......（后略) ......
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