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.

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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