1 ANEMIA Tongji Medical College, Huazhong University of Science and Technology.
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Transcript of 1 ANEMIA Tongji Medical College, Huazhong University of Science and Technology.
1
ANEMIA
Tongji Medical College, Huazhong University of Sci
ence and Technology
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Introduction
Anemia is a commonly encountered clinical symptom that is caused by an acquired or hereditary abnormality of red blood cells (RBC) or its precursors, or may be a manifestation of an nonhematologic disorder
hematologic adj.
hematology n.
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Definition
Anemia is defined as a decrease in the circulating RBC or hemoglobin and a corresponding decrease in the oxygen-carrying capacity of the blood.
Normal values of the hemogram:
TEST WOMEN MEN HCT (%) 37-43 42-49 Hb (g/L) 11.3-13.5 12.7-15.3 RBC Count(1012/L) 3.83-4.83 4.29-5.58 HCT:Haematocrit Hb:hemoglobin
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Definition
A decrease in any of these values (HCT, Hb, RBC count) is called anemia.
TEST WOMEN MEN
HCT (%) < 37 < 42
Hb (g/L) < 110 < 120
RBC Count(1012/L) < 4.0 < 4.5 They can be altered by the plasmatic volumes .Difference between women and men values are due to androgen hormones.For pregnant women, Hb < 100g / L
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cell morphological classification
cell morphological type MCV ( fl ) MCHC (%)
Macrocytic anemia > 100 32 ~ 35Normocytic anemia 80 ~ 100 32 ~ 35Microcytic hypochromic anemia < 80 < 32
MCV : mean corpuscular volume ;
MCHC : mean cell hemoglobin concentration
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Macrocytic anemia
•Megaloblastic anemias ( folic acid and vitamin B1
2 deficiency) •Alcoholism •Drugs •Liver diseases •Primary bone marrow disorder •Hypothyroidism •Splenectomy
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Normocytic anemia
•Chronic disease anemia (hepatic, renal or endocrine disorders)
•Primary bone marrow disorders (aplasia, myelodisplasia, myelofibrose,hematologic and solid tumors, HIV infection, granulomas•Hemolytic anemia
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Microcytic anemia
•Iron Deficiency
•Thalassemia
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Classification according to severity
Degree Hb ( g/L )
Mild anemia 90 ~ 120 Moderate anemia 60 ~ 90Severe anemia < 60Very Severe anemia < 30
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Classification: according to Bone marrow proliferative degree
Bone marrow anemia
hyperplasia IDA, hemolytic anemia, blood loss anemia
Hypoplasia AA, PRCADysmaturity(ineffective erythropoiesis) myelodysplastic syndrome(MD
S)
Megaloblastic anemias
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Classification: according to etiology/pathogenesis
etiology/pathogenesis
Decreased RBC production Increased RBC destruction Blood loss
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Classification: according to etiology/pathogenesy
Decresed RBC production Anemia
1. BM stem cells abnormality AA2. BM injured by abnormal tissues/cells Leukemia3. Cell dysmaturity (1). DNA dyssynthesis Megaloblastic anemia
s (2). Hb dyssynthesis IDA4. Abnormality in haematopoiesis modulation AA
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Classification: according to etiology/pathogenesis
Increased RBC destruction Anemia
1. RBC internal defect(1)RBC membrane defect acquired: PNH hereditary : hereditary spherocytosis(2) enzyme deficiency: G6PD deficiency (3) Hb abnormality hemoglobinopathy(4) porphyrin metabolism porphyrinopathy
2. Exopathic immunologic, chemical, biological,
or phisical factors
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Classification: according to etiology/pathogenesy
Blood loss
1. Acute blood loss2. Chronic blood loss
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Related factor;
1. cause of anemia
2. Degree of decrease of oxygen carrying capacity
3. Degree of decrease of blood volume
4. Speed of anemia genesis
5. Compensation and tolerance of blood and other
Clinical manifestation
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1 、 nervous system :headache 、 dizzy 、 dispirited 、 faint 、 ear noises 、 dim eyesight 、memory impairment 、 impaired concentration ; acroanesthesia , intel
ligence development abnormality
2 、 skin and mucosa : pallor ; rough ; ulcer, stained yellow, Koilon
ychia
3 、 espiratory apparatus : accelerated breathing ; short breath 、 4 、 digestive system : abdominal distention 、 anorexia, bowel distur
bance, swallow foreign body sensation ; Decreased food appetite; mir
ror surface tongue
Clinical manifestation
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5 、 urinary system : bilirubinuria 、 hemoglobinuria 、
urinary siderosis ; ARF
6 、 circulating system : peripheral vaso-constriction ,
Palpitation, congestive heart failure
7 、 endocrine system : endocrine functional abnormality
8 、 immune system :
Clinical manifestation
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9 、 hematological system :
peripheral blood:
blood cell count, appearance and biochemical component ;
plasma ,serum
hematopoietic organ :BM , liver , spleen, lymph node
10 、 genital system :
Clinical manifestation
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complete diagnosis
1. anemia
2. Degree of anemia
3. Classification of anemia
4. Cause of anemia
Diagnosis
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History(inquisition):
1. Present history(symptoms and their duration)
2. Past history(Gastrectomy, haemorrhoids, peptic ulcer)
3. Family history(hereditary background)
4. Menses and childbearing history
5. Diet habit
6. Exposure to risk factors(such as chemicals or toxins)
Diagnosis: history
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Diagnosis: Physical examination
1. Pale (lip, palpebral conjunctiva, finger nail )
2. accompanying symtoms
hemolytic
bleeding
infiltration
infection
malnutrition
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laboratory investigation :
1. Peripheral blood(PB) (1)blood routine test : RBC, HCT,Hb,
Erythrocyte indexes(MCV,MCH,MCHC)
(2)blood smears
(3)Reticulocyte count(Ret)
2.Bone marrow(BM) aspiration
3.investigation for cause:
urine routine test, renal function test, Occult blood in stools,
X ray or endoscope for stomach and bowel
Diagnosis: laboratory investigation
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1. Treatment for original cause
2. Treatment for symptoms
transfusion
haemostasis
anti-infection
supportive therapy
Treatment
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Break
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Aplastic anemia
(AA)
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Definition
The term aplastic anemia describes a clinical syndrome in which there is a deficiency of red cells,neutrophils, and monocytes,and platelet without mophological evidence of another marrow disorder. Marrow examination shows a near absence of normal hematopoietic precursor cells and fatty replacement .
It is a life-threatening hematologic disease.
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Definition
The term pancytopenia
If all of the cell lines(erythrocytic, leukocytic, and thrombocytic) are affected and decrease, the disorder is referred to as pancytopenia.
If only one cell line is involved, it is usually the erythrocytic cells. (pure red cell anemia,PRCA)
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Etiology and pathogenesis
Etiology
1. Idiopathic about 65% of all cases
2. Acquied (1)chemicals Benzene(2)drugs chloramphenicol(3)radiation(4)viruses Epstein-Barr virus, hepatitis virus(5)miscellaneous pregnansy , connective tissue disorders
3.Hereditary
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Etiology and pathogenesis
Pathogenesis
1. “Seeds”
Absent or defective stem cells
2. “Soil”
Abnormal bone marrow microenviroment
3. “Worms ”
Abnormal regulatory cells or factors
Immue-mediated supression of hamatopoiesis
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Clinical manifestation and classification
Clinical manifestation
• Anemia normochromic normocytic
• Bleeding
• Infection
Classification
1.severe aplastic anemia ( SAA ) : type I type II
2.non-severe aplastic anemia ( NSAA )
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1.peripheral blood: pancytopenia
normochromic and normocytic anemia
SAA:( 1 ) Hb < 30g/L; reticulocyte<1 %, absolute value < 15×109 / L 。( 2 ) neutrophil < 0.5×109 / L( 3 ) platelet < 20×109 / LVery severe aplatic anemia(VSAA ): SAA-Ⅰ neutrophil < 0.2x109/L.NSAA :( 1 ) Hb 40 ~ 60g/L , Ret > 1 %, its absolute value is lower than no
rmal.
( 2 ) neutrophil > 0.5×109 / L , relative value of lymphocyte increas
es.
( 3 ) BPC(blood platelet count) > 20×109 / L
Laboratory findings
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2 、 Bone marrow smear
Bone marrow hypoplasia
Hematopoietic cells decrease so proportion of non hemat
opoietic cells (lymphocyte, monocyte, plasmacyte, basophi
l ) increases.
Platelet is rare and NAP count increases.
SAA no megakaryocyte
NSAA residual areas of hematopoiesis(hot spot)
megakaryocyte decreased or absence
Laboratory findings
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3 、 Bone marrow biopsy : better than smear
Red pulp is replaecd by fatty tissues. Hematopoietic cells is less than half o
f normal normal situation , ratio of fatty tissues increses. Megakaryocyte i
s rare or absent.
4 、 Bone marrow ECT :
It helps to evaluate remained hematopoietic activity .
Laboratory findings
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1.pancytopenia, reticulocyte absolute value decreases.2.no splenormegaly
3.at least on area of hypoplasia in bone marrow(significant reduction
of megakaryocytes is needed if hyperplasia is found ). Non-hematop
oietic cells increase .
4. Elimination of other diseases which may cause pancytipenia such a
s PNH, MDS,MFMyelofibrosis, acute arrest of hemopoiesis,etc.5. Common therapy for anemia is ineffective.
Diagnosis
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Differentiation between SAA and NSAA
SAA NSAAProgress acute chronicbleeding severe mild often occurred in internal organ often occurred in skin and mucosa
infection severe sepsis mild Upper respiratory tract
PB ( ×109/L ) Neutrophil < 0.5 > 0.5 BPC < 20 > 20 Ret absolute value < 15 > 15BM most areas of hypoplasia hypoplasia or hyperplasia Prognosis bad, die in 6 to 12 months better , a few patients may die
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1 、 paroxysmal nocturnal hemoglobinuria ( PNH )
2 、 myelodysplastic syndrome ( MDS )
3 、 hypoproliferative leukemia
4 、 acute arrest of hemopoiesis
5 、 Myelofibrosis
6、 autoantibody mediated pancytopenia
Differential diagnosis
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1 、 withdrawal of etiologic agents ( acquired AA )2 、 supportive care : ( 1 ) anemia : RBC transfusion(Hb < 60g/L )
( 2 ) bleeding : BPC<20×109 / L, spontaneous bleeding
platelet transfusion ( 3 ) infection :
1aminar flow ward, cleaning of skin,mouth cavity,anus and diet Antibiotics used early and strongly Neutrophil transfusion is not recommended
Therapy
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3 、 SAA
( 1 ) hematopoietic stem cell transplantation(HSCT) : allo-HSCT
( 2 ) Immunosuppressive therapy :
a . antilymphocyte globulin ( ALG ) or antilymphocyte globulin ( AT
G )
b. High dose methylprednisolone ( HDMP )
C . cyclosporin A ( CsA )
Combination of ATG/ALG, CsA and HDMP
(3) Hemopoietic growth factor:
EPO, granulocyte colony-stimulating factor (G-CSF), IL-11
Therapy
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4 、 NSAA a . androgen : b . CsA c . Other drugs : Securinine 、 Levamisole
d . combination of traditional Chinese medicine and
western medicine
Therapy
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Thank you