hemat merge

Anemia Outline

• Anemia
  • Basic concept, clinical manifestation, main laboratory examination
  • Diagnosis procedure, etiological diagnosis, therapeutic principle
  • Classification

Contents

• Definition
• Pathogenesis
• Clinical manifestation
• Laboratory examination
• Diagnostic method
• Therapeutic Principle

Anemia Definition

• Lower than lower limits of normal for the same sex, age, district in:
  • Hemoglobin
  • Hematocrit value
  • Red blood cell count
• Anemia is a syndrome, not a disease

Normal Values

• Normal value for peripheral blood in adults:
  • Male:
    • Hb: $130-160 \, g/L$
    • RBC count: $4.5-6.0 \, 10^{12}/L$
  • Female:
    • Hb: $120-150 \, g/L$
    • RBC count: $3.9-5.0 \, 10^{12}/L$
  • Both:
    • MCV: $80-100 \, fl$
    • MCH: $27-33 \, pg$
    • MCHC: $32-35 \,$
    • WBC: $4.0-10.0 \, 10^9/L$
    • Platelets: $100-300 \, 10^9/L$
    • Reticulocytes: $0.5-1.5 \,$

RBC Classification

• Diagnosis of anemias by RBC indices:
  • Small cells (microcytes): Low MCV ($<80 \, fl$), Normal MCHC ($<32 \,$) -> Iron deficiency
  • Normal-sized cells (normocytes): Normal MCV ($80-100 \, fl$), MCHC ($32-35 \,$)
  • Large cells (macrocytes): High MCV ($>100 \, fl$) -> VitB12 or folate deficiency, Aplastic anemia

Pathogenesis

• Erythropoiesis decrease
  • Hematopoietic stem cell abnormity: Aplastic Anemia, Hematologic Malignancies
  • Hematopoiesis accommodation abnormal
  • Hematopoiesis raw material deficiency
• Hyper-hemocatheresis: hemolytic anemia
• Blood loss anemia

Clinical Manifestation

• Relevant to cause, degree, emergency, tolerance
  • Nervous system
  • Skin and mucous membrane
  • Circulatory system
  • Respiration digestion. Urology, endocrine reproduction, immune, hematological system.

Laboratory Examination

• Blood routine, blood platelet count
• Reticulocyte
• Bone marrow puncture
• Bone marrow biopsy
• Pathogenesis detection:
  • iron metabolism
  • serum folic acid and VitB12 level

Diagnostic Method

• Case history
• Physical examination
• Laboratory examination

Therapeutic Principle

• Etiological treatment
• Pathogenetic treatment
• Symptomatic treatment
• Composition blood transfusion

Thinking Points

1. How to diagnose and treat an anemia patient?
2. Why is etiological diagnosis very important?

Aplastic Anemia

• Aplastic Anemia
  • Clinical manifestation and hematologic characters
  • Diagnosis basement and Differential diagnosis, treatment
  • Etiological factors, the mark of bone marrow Pathology
  • Pathogenesis

Contents

• Definition
• Etiological factor
• Pathogenesis
• Clinical manifestation
• Laboratory examination
• Diagnostic criteria
• Differential Diagnosis
• Treatment

Definition

• Acquired bone marrow blood-producing function failure
• Characteristic: Hypo-bone marrow blood-producing function, pancytopenia
• Clinical feature: anemia, bleeding, infection
• Effective with immunosuppressive agents
• Classification
  • severe aplastic anemia, SAA
  • non-severe aplastic anemia, NSAA

Etiology

• Not identified, but maybe:
  • Infection viral: hepacivirus, human parvovirus B19
  • Chemical factor: chloramphenicol, benzene
  • Radial line
  • Immunologic abnormality

Pathogenesis

1. Progenitor cells defect
   • CD34+ cells↓
2. Injury of hematopoietic microenvironment
   • Marrow stroma cell cultivation
3. Immunolesion of bone marrow
   • Mediated by T cells
   • Th₁ cells
   • CD8+T suppressor cells↑
   • CD25+T cells γδTCR+T cells↑
   • IL-2 IFN-γ, TNF↑

Clinical Manifestation

• Anemia progress rapidly
  • SAA
  • Infection, fever
  • NSAA
  • Bleeding generally, risk for intracranial hemorrhages

Laboratory Examination

• Hemogram
  • Sever Pancytopenia in SAA
    • WBC $<2 \times 10^9 / L$
    • $[N]<0.5X10^9/L$
    • PLT$<20 \times 10^9/L$
    • Reticulocyte absolute value$<15 \times 10^9/L$
  • NSAA: can not attain SAA degree
• Bone marrow image
  • The bone marrow aspirate and the bone marrow biopsy appear hypocellular, with only scant amounts of normal hematopoietic. No abnormal cells are seen.
• SAA blood: red blood cell morphology was normal, only see a white blood cell. Few platelets
• NSAA blood: red blood cell morphology was normal, lymphocytes, neutrophils and platelets
• SAA myelogram: nuclear cell proliferation of severe reduction
• NSAA myelogram: nuclear proliferation to reduce also, but NSAA's blood cell is more than SAA'blood.
• NSAA bone marrow:
  • lymphocytes are more visible, neutral metamyelocyte .Rod-shaped nucleus and segmented neutrophils, immature red nucleus highly dense, concentrated was " carbon core" sample
• SAA bone marrow:
  • Bone marrow Island was empty net, only to see the fibroblasts (1), Lymphocytes and a large number of reticular fibers, no hematopoietic cells
• Bone marrow biopsy
  • AA bone marrow (filled with adipose tissue)

Diagnostic Criteria

• Pancytopenia
• Reticulocyte absolute value↓
• No liver, spleen and lymph nodes swelling generally
• Hyperplasia in most part of bone marrow↓, hematopoietic tissue↓
• Except for other pancytopenia

Differential Diagnosis

• With other Pancytopenia
  • Paroxysmal nocturnal hemoglobinuria (PNH)
  • Myelodysplastic syndrome (MDS)
  • Leukemia
  • Autoantibodies Mediated Pancytopenia
  • Malignant histiocytic disorder
  • Acute blood-producing function stasis
  • Fanconi's anemia

Treatment

1. Supporting Therapy
   • Infection prevention
   • Bleeding prevention: Blood Platelet Transfusion
   • Rectify anemia: blood composition transfusion
2. Symptomatic treatment
   • Control infection
   • Aid to liver
3. Immunosuppressive therapy
   • ALG/ATG: horse ALG $10\sim15mg/kg\times5day$
   • rabbit ATG $3\sim5mg/kg \times5ay$
   • Ciclosporin A: $3\sim5mg/(kg.d)$
   • Other: methylprednisolone
4. Promote hematogenesis
   • Hormonal therapy
     • Androstanazol 2mg tid
     • Andriol 40mg tid
   • Hemopoietic growth factor
     • GM-CSF, G-CSF, EPO
5. Hematopoietic stem cell transplantation
   • Below 40y
   • No other complication
   • Can be considered Suitable donor SAA

Thinking Points

1. Diagnostic criteria and classification
2. Therapeutic principle and methods
3. Differential diagnosis for pancytopenia.

Hemolytic Anemia

• Hemolytic Anemia
  • Diagnose steps
  • Clinical manifestation
  • Laboratory examination

Contents

• Definition
• Pathogenesis
• Clinical manifestation
• Laboratory examination
• Diagnostic method
• Therapeutic Principle

Definition

• Hemolytic anemia: red blood cell survival is reduced, the ability of the bone marrow to compensate is outstripped.
• Hemolytic disease: hemolysis, the bone marrow increase erythroid production (6-8 fold) in response to reduced red cell survival.

Etiology

• Red blood cell intra-cellular defect
  1. Heredity:
     1. Membraneous defect: hereditary spherocytosis
     2. Enzyme defect: G6PD deficiency
     3. Globin abnormality :Mediterranean anemia
  2. Acquired: paroxysmal nocturnal hemoglobinuria, PNH
• Extrinsic factor
  1. Immunity:
     1. Autoimmune Hemolytic Anemia, AIHA
     2. Isotype immune hemolytic anemia
        1. Hemolytic disease of newborn
        2. Blood transfusion reactions by Blood Group Incompatibility
  2. Physical and machinery injury:
     1. March hemoglobinuria, Artificial heart valve
     2. A large area burns、 radiation injury
  3. Chemicals and biological factor:
     • Nitrite poisoning, snake poison

Clinical Manifestation

1. Acute hemolytic anemia:
   1. Headache, vomiting, hyperpyrexia
   2. Aching pain on lumbus, back and extremities, abdominal pain
   3. Urine as soy color
   4. Pale face and jaundice
   5. Peripheral circulatory failure
2. Chronic hemolytic anemia:
   1. Anemia
   2. Jaundice
   3. Hepatosplenomegaly

Normal Red Blood Cell Metabolism

• Hemoglobin is broken down into hemobilirubin by mononuclear macrophages.
• Hemobilirubin combines with glucuronate to form conjugated bilirubin.
• Conjugated bilirubin is converted to urobilinogen in the portal vein.
• Urobilinogen is further metabolized into urobilin and stercobilinogen.

Laboratory Examination

1. Determine whether this is a hemolytic anemia
   1. Red blood cell survival Shortening:
   2. increased red cell breakdown:
      • RBC↓, Hb↓, without bleeding Extravascular hemolysis check
        1. Hemobilirubin ↑
      • Urobilinogen discharge ↑ ‚urine bilirubin(-)
        1. Stercobilinogen ↑
      • Intravascular haemolysis check
        1. Hemoglobinemia
        2. Urinary siderosis
        3. Serum haptoglobin reduced
        4. Urinalysis: positive for occult blood and urine protein, erythrocyte- negative
   3. erythrocytes compensatory hyperplasia
      1. Reticulocytosis, absolute value↑
      2. Erythroblasts in Peripheral blood.
      3. Erythroblasts proliferation in bone marrow
   4. Erythrocytes morphologic abnormality:
      5. polychromasia, Howell-Jolly body, cabot loop
2. Determine hemolytic causes further
   1. Coombs test (AIHA)
   2. Ham test positive (PNH)
   3. Hemoglobin electrophoresis: thalassemia
   4. Isopropanol test: unstable hemoglobinopathy
   5. Methemoglobin reduction test and Heinz body production: G6PD deficiency
   6. Particular form: target cell
   7. Osmotic Fragility: hereditary spherocytosis

Diagnosis

• Clinical manifestation
  1. Case history
  2. Signs and symptoms
• Laboratory examination

Treatment

1. Remove the causes and inducement
2. Glucocorticoids: AIHA
3. Immunosuppressive agents: AIHA
4. Splenectomize:
5. Transfusion:

Autoimmune Hemolytic Anemia, AIHA

• Caused by red cell autoantibody
• Clinical typing
  • Warm antibody AIHA-37 °C
  • Cold antibody AIHA-4°C

Warm antibody AIHA

• Red blood cell destruction is caused by autoantibodies, which have the most affinity to erythrocyte antigens in temperatures of 37 degrees Celsius.
• Mainly IgG autoantibodies Warm antibody AIHA is the most common type, can be seen at any age, with more young and middle-aged women
• Etiology
  • Primary: unknown origin
  • Secondary: Lymphoma, chronic lymphocytic leukemia, SLE, infection, cancer, drugs.
• Clinical manifestation
  1. Slow progression: dizziness, weakness, anemia, recurrent attacks
  2. Anemia: as major symptoms
  3. Jaundice: liver and spleen can be mild to moderate swelling called Evans syndrome
  4. Secondary:
     • Performance of the primary disease, easy to overlook this disease
  5. Acute illness: which mainly occurs in children, and occasionally in adults, acute hemolytic
  6. Special types: AIHA partners with thrombocytopenic purpura, called Evans syndrome
• Laboratory Examinations
  1. Hemoglobin decreased, degrees is varying; reticulocytes increased; blood smear: spherocytosis and larger red blood cells and nucleated red blood cellsis visible.
  2. the red blood cells in bone marrow hyperplasia
  3. Serum bilirubin: usually about $42.75-85.50\,μmol/L$ (2.5mg\%\sim5mg\%$), dominated by indirect bilirubin
  4. Anti-human globulin test (Coombs test): direct-positive. Few Coombs test is negative, but the hormone therapy effective
• Treatment
  1. Get rid of the etiology
  2. Glucocorticoid :
     • The first choice for warm antibody AIHA.
  3. Immunosuppressive drugs:
     • Treatment with glucocorticoid is invalid or has to rely on high doses of prednisone to maintain, or invalid after Splenectomy or relapse.
  4. Splenectomy
  5. Support
     • Transfusion of washed red blood cells:
       • Transmission of whole blood can provide a large amount of complement, aggravating the risk of hemolysis.
       • When serious life-threatening anemia, transfusion of washed red blood cells, must be carefully look at patients
     • Folic acid supplementation: due to bone marrow hematopoietic strong, relatively insufficient folic acid

Paroxysmal nocturnal hemoglobinuria (PNH)

• PNH is a result of hematopoietic stem PIG-A disease caused by gene mutations, characterized by acquired erythrocyte membrane intrinsic defects of chronic intravascular hemolytic anemia, primarily clinically characterized by chronic intravascular hemolysis and hemoglobinuria
• Diagnosis
  • Clinical manifestation
    1. Chronic intravascular hemolysis, intermittent Hb in urine (urine color of soy sauce)
    2. Dominated by anemia, symptoms of thromboembolism, infection
    3. Aplastic anemia -PNH syndrome
• Laboratory tests
  1. Blood: hemoglobin reduced, pancytopenia
  2. Bone marrow: erythroid hyperplasia
  3. Acidified serum hemolysis test (Ham test): +
  4. Sucrose hemolysis test: +
  5. Venom factor (cobra venom factor, CoF) hemolysis test: +
  6. Urinary hemosiderin (Rous test): +
  7. Membrane protein abnormality detection: CD55 and CD59 deficiency
• Treatment
  1. The incentive for the prevention of acute hemolytic episode
  2. Acute hemolysis treatment:
     1. Remove incentives
     2. Glucocorticoids
     3. 6% dextran 500 \sim 1000ml intravenous infusion
     4. Prevention of acute renal failure: alkaline urine
     5. Prevention of thrombosis
  3. Treatment of anemia: androgens, folic acid, iron supplements, infusion washed red blood cells
  4. Allogeneic bone marrow transplantation

Exercises (Hemolytic Anemia)

1. During extravascular hemolysis, the main site of destruction of red blood cells is the spleen.

2. The most valuable splenectomy for hemolytic anemia is hereditary spherocytosis, or primary autoimmune hemolytic anemia that does not respond to glucocorticoid therapy

   Case study

   • Female, 30 years old with SLE:
     • Most likely lab test with abnormal results is Coombs test.
   • Female, 20 years old:
     • Preferred treatment is glucocorticoids.

Review Questions

1. How to diagnose a hemolytic anemia in an anemic patient?
2. How to use component transfusion cure hemolytic anemia correctly?

Iron Deficiency Anemia (IDA)

• Iron Deficient Anemia (IDA)
  • Cause of disease, Clinical manifestation and Laboratory examination, Diagnosis main points, Differential Diagnosis, Therapeutic Principle and measures
  • Iron metabolism
  • get the message the incidence and prophylaxis

Contents

• Definition
• Iron metabolism
• Causes
• Clinical manifestation
• Laboratory examination
• Diagnostic criteria
• Differential Diagnosis
• Therapy

Definition

• IDA develops when there is inadequate iron for hemoglobin synthesis.
• Iron deficiency:
  • iron depletion, ID↓intra-red cellular iron deficiency
  • iron deficiency erythropoiesis, IDE↓iron deficient anemia, IDA

Normal Iron Metabolism

• Body distribution
  • For health adult, iron volume dose 2\sim4g
    • Hemoglobin, most of the iron
    • Myoglobin
    • Enzyme (0.2\sim0.4g)
    • Bone marrow
    • Liver (0.5\sim2g)

Normal Iron Absorption

• Occurs in the Duodenum and upper jejunum.
• Fe^{3+}$is converted to$Fe^{2+} by reductases.
  • This is then kept in storage or utilized with the use of transporter.

Causes

1. Increased demand for iron and/or hematopoiesis
   • Rapid growth in infancy or adolescence, Pregnancy, Erythropoietin therapy
2. Increased iron loss
   • Chronic blood loss, Menses, Blood donation, Phlebotomy as treatment for polycythemia vera
3. Decreased iron intake or absorption
   • Inadequate diet, Malabsorption from disease(sprue, Crohn's disease), Malabsorption from surgery(post-gastrectomy), chronic inflammation.

Clinical Manifestation

1. Primary diseases
2. General anemia
   • Easy fatigability, palpitations and tachypnea on exertion.
3. Tissue iron deficiency
   • Severe deficiency causes skin and mucosal damage.
   • Smooth tongue
   • koilonychia
   • Sideropenic dysphagia (plummer-Vinson syndrom)
   • Nerve or psyche system abnormal: pica-craving for specific foods(ice chips,etc)

Laboratory Examination

• Hemogram:
  • MCV decreased
  • Mature red blood cells range in size, light-stained area of the Centre expanded.
• Blood Routine
  • Midrange anemia, MCV decreased
• Bone marrow picture:
  • Erythroblasts "older nuclei and younger plasm"
  • Active proliferation of the red blood cell system, dominated by, late immature red blood cells. Immature red blood cell volume reduced, nuclear chromatin of tight, little cytoplasm, blue, ragged edge.
• Iron metabolism
  • Serum iron( SI) <8.95\,μmol/L
  • Total iron binding capacity( TIBC)>64.4\,μmol/L
  • Transferrin saturation(TS)<15\%$Serum ferritin (SF)$<12µg/L$
• Iron stain in bone marrow slides
  • Extracellular iron -negative, bone marrow extracellular matrix without iron particles. ( Positive reaction bluish green precipitate)
  • Bone marrow iron stain: intracellular iron is negative, no iron particles immature erythrocytes. ( Positive reaction was blue and green granular)

Etiological Diagnosis

• Only Identify the cause, Can IDA be cured.

Differential Diagnosis

• Small cell anemia include the following:
  1. Sideroblastic anemia
  2. Thalassemia
  3. Anemia of chronic disease, ACD

Treatment For IDA

• Eradicate the etiological factors, Supply enough storage
  1. Etiological treatment
  2. Supply chalybeate
• Supply Chalybeate
  • Take orally as first choice
    • Representative: ferrous sulfate, 3-6months after HB is normal
  • Indication for injections
    • Commonly used: iron dextran
    • Intramuscular to deep part, and note allergy

Exercise

• The earliest indicator of the effectiveness of iron in the treatment of iron deficiency anemia is Elevated reticulocyte

• Iron is absorbed at Duodenum and jejunum superior

  Case Study

  • Female, 25 years old:
    • The most fundamental treatment is Iron was given
  • Pregnant woman, 30 years old:
    • The substance that should be supplemented is Animal liver, meat

Thinking

1. Experiments Indexes For IDA
2. Chalybeate Therapeutic Principle

Disseminated Intravascular Coagulation (DIC)

• Disseminated Intravascular Coagulation (DIC)
  • Diagnosis and treating principle.
  • Clinical manifestation, main diagnosis index in laboratory findings.
  • Causes and pathogenesis.

Contents

• Definition
• Etiology
• Pathogenesis
• Clinical manifestation
• Diagnosis criteria
• Differential diagnosis
• Treating

Definition

• A clinical syndrome caused by etiological factor activating blood clotting and fibrinolysis systems, which inducing microthrombosis all over the body, blood coagulation factors consumed massively, secondary hyperfibrinolysis, bleeding and circulatory failure.

Etiology

• Infection: main cause
• Malignant neoplasm: AML-M3,liver cancer, lung cancer
• Pathological gynecology and obstetrics:
• Trauma:
• Others:

Pathogenesis

1. Tissues、vascular endothelium、platelet injury and fibrinolysis activating
2. Microthrombosis、coagulation disorder microcirculation disturbance

Clinical manifestation

• Bleeding: spontaneous multiple, general
• Shock: circulatory failure
• Embolism: microthrombus widespread, most in kidney, lung and brain
• Hemolysis: Microangiopathy
• Primary diseases

Diagnosis criteria

• Clinical manifestation
• Basis disease
• Above 2 items of the following:
  • Multiple bleeding
  • Inexplicable shock
  • Multiple capillary embolism
  • Effective for anticoagulant therapy

Laboratory examination index

• Platelet $<100\times10^9/L$
• Fibrinogen$<1.5g/L$ or $>4 g/L$
• FDP$>20mg/L$, or D-D high
• PT shorten or longer than 3s (liver ailment>5s) ;
• APTT shorten or longer than 10s

Differential diagnosis

• Severe liver disease
  • Liver function damaged severely
• TTP (thrombotic thrombocytopenic purpura)
  • Fever bleeding with thrombocytopenia
  • Microangiopathy haemolysis signs on central nervous system, kidney injury
• Constitutional hyperfibrinolysis
  • D-Dimer (-)

Treatment

• Removal the causes and treat the underlying disease
• Anticoagulant therapy
  • Common heparin (uFH): APTT as monitoring test, APTT at $1.5\sim2.5$ double of normal value; drug should be discontinued if platelet count lower than $50 × 10^9 / L$.
  • Low molecular weight heparin (LMWH): drug should be discontinued if platelet count lower than $50 × 10^9 / L$.
• Supply platelet and coagulation factors
  • Platelet$(>(20\sim50)\times10^9/L)$
  • Fresh frozen plasma fibrinogen, Prothrombin Complex
• Antifibrinolysis
• Thrombolysis
• Others

Exercise

• Male, 5 years old:
  • The patient's most likely diagnosis is Pulmonary infection with DIC
• DIC consumptive low coagulation period is preferred heparin

Thinking

1. Etiological factors?
2. Diagnosis and treatment ?

Hemorrhagic Disease

• Hemorrhagic Disease
  • Diagnosis and laboratory screening tests
  • Normal hemostatic mechanisms
  • Classification and treatment for bleeding disease

Contents

• Overiew
• Etiology and pathogenesis
• Clinical manifestation
• Laboratory findings
• Diagnosis criteria
• Treatment

Overview

• Hemostatic function defect Autogenous hemorrhage or haemophilia after injury of blood vessel skin mucosal bleeding

Hemostatic mechanisms

• Normal haemostasis
• Normal blood clotting
• Anticoagulating and fibrinolysis mechanism

Normal haemostasis mechanism

• Vascular elements
• Platelet mechanisms
  • Platelet adhesion mediated by GPIb von willebrand factor, vWF
  • Platelet aggregation and granule release

Normal coagulating mechanism

• Blood clotting agents
  • Blood coagulation factors
    • I、 II、 III、 IV (Ca2+ ), V、 VII、 VIII, IX, X, XI、 XII XIII PK(prekallikrein) HMWK (high molecular weight kininogen)
    • Thromboplastin
    • Thrombin
    • Intrinsic coagulation pathway
    • Extrinsic coagulation pathway
    • Fibrinogenesis

Etiology and pathology

• Hemorrhagic diseases are classified as:
  • Abnormality of vessel wall
  • Abnormality of platelet
  • Abnormality of coagulating

Abnormality of blood vessel wall

• Congenital or inherit hereditary telangiectasia; familial purpura simplex; congenital connective tissue disease
• Acquired infection; allergy; chemical materials and drugs; malnutrition; dysendocriniasis; others

Abnormality of platelets

• Quantitative platelets disorders
  • Thrombocytopenia
    1. Thrombopoiesis: AA. Leukemia
    2. Excessive Platelet destruction: ITP
    3. Excess consumption: DIC
    4. Abnormality of distribution: hypersplenism
  • Thrombocytosis
    • Primary
    • Secondary cases
• Qualitative platelets disorders.
  • Heredity: thrombasthenia, Giant Platelet Syndrome, thrombocytopathy
  • Aquired: antiplatelet drug infection uremia globulinemia

Abnormality of coagulating

• Congenital or inherited disease
  • HemophiliaA, B and inherited FXI deficiency
  • Genetic prothrombin、 FV、 VII. X deficiency, genetic fibrinogen deficiency genetic FXIII deficiency or penia
• Acquired
  • Liverish coagulation disorder
  • Vitk dificiency
  • uremia

Abnormality of anticoagulation and fibrinolysis

• Acquired disease in general
  • Over dose of heparin
  • Coumarins overdose and sodium diphacinone intoxication
  • Anti-VIII, IX antibody formation
  • Snake bites, leech bites
  • Thrombolytic drug overdose

Compound hemostasis mechanism disorders

• Congenital von willebrand disease(vWD)
• Acquired disseminated intravascular coagulation(DIC)

Clinical manifestation

• Bleeding
• Relevant disease signs
• General signs:
  • Heart rate
  • Respiration
  • Blood pressure
  • Distal circulation status

Clinic differential for common hemorrhagic disease

Laboratory findings

• Screening tests (table)
  • Abnormality of blood vessel wall
    • BT, CFT (capillary fragility test)
  • Platelets abnormal
    • BPC (blood platelet count) -
    • Clot retraction test. BT, CFT
  • Disorders of hemostasis
    • Fg PT APTT TT

Confirmed diagnosis test

• Abnormality of blood vessel wall
  • Capillaroscope vWF, ET-1
• Platelets abnormal
  • Shape, functions, PF3 PAIg TXB2
• Disorders of hemostasis
  • Factor V VII, VII, IX, X, XI, XII thrombin II F1+2 fibrinogen I
• Anticoagulation abnormal
  • AT, FVIII:C-antibody
• Fibrinolysis
  • FDP, D-D, PLG, t-PA Special test

Diagnosis criteria

• Make rudiment judgement by case history (family history and basic diseases) and clinical manifestation
• congenital or acquired
• Make initial differential by clinical manifestation
• vascular platelet and coagulation disorder Identify by screen test vascular platelet and coagulation disorder Confirmed diagnosis test vascular platelet and coagulation disorder

Treatment

• Prevention and cure of causes
  • Basic disease, avoid anything inducing abnormality of hemostasis or clotting
• Hemostasis treating
  • Supply platele or coagulation factors drug: Vitk, TPO IL-11
• Other treatment
  • Anticoagulation: heparin plasma exchange surgery chinese herbal drug gene therapy

Exercise

• The main purpose of platelet infusion is Improve hemostatic function
• Male, 18 years old with Hemophilia A Then the deficient factor in this patient is Factor VIII lacks
• Among the following clotting factors that are not vitamin K-dependent are Factor VIII

Thinking

• How to distinguish the