Chapter 11.

General Consideration of
Hemorrhagic Disease
 Hemostatic mechanism

Blood vessel factors: vWF

Coagulation factors

Platelet factors
   Classification of hemorrhagic disease

1. Vascular disorders
Hereditary : hereditary telangiectasia
Acquired: hypersensitivity purpura
2. Platelet disorders
Thrombocytopenia :
Decreased platelet production: aplastic anemia
Increased platelet destruction:
idiopathic thrombocytopenic purpura
Platelet depletion:
disseminated intravascular coagulation.
Sequestration of platelet: hypersplenism.
Thrombocytosis:
Primary thrombocytosis
Qualitative platelet disorders
3. Disorder of blood coagulation
Hereditary: hemophilia
Acquired: vitamin K deficiency
4. Disorder of anticoagulation and fibrinolysis
Excessive heparin
 Chapter 12

Idiopathic Thrombocytopenic Purpura(ITP)

= Immue Thrombocytopenia

autoimmune disorder

IgG autoantibody is formed

Platelet destroyed in the spleen

·        Clinical findings
Acute ITP Chronic ITP
Age childhood young female
Cause frequently viral infrequently viral
infection infection
Illness rising acute chronic
Bleeding skin, mucosal, purpura, petechiae,
internal organs menorrhagia
intracranial hemorrhage
Illness course usually self-limited long, often recur
seldom recur
PLT count < 20 × 109/L 50 × 109/L
PLT survival 1-6hr 1-3d
·        Laboratory findings
1.  thrombocytopenia , occasional anemia, platelets are
slightly enlarged. These larger platelet are young platelets
produced in response to enhanced platelet destruction.
2.  The bone marrow :
Increased marrow megakaryocytes with a shift to
younger, less polyploid megakaryocytes and fewer mature, less
platelet-producing megakaryocyte
3.  platelet-associated IgG(PAIgG) and C3(PAC3)
80% patients is high
·        Diagnosis

1.   Present bleeding

2.   Isolated thrombocytopenia
3.   No splenomegaly or slight splenomegaly
4.   BM: megakaryocytes increase and immature
5.   One of the following conditions
Respond to prednisone, splenectomy is effective,
PAIgG(+), PAC3(+), platelet survival shorten
·        Treatment
  1.Prednisone
Mechanism : (1) Decreasing the affinity of splenic
macrophages for antibody-coated platelet.
(2) Reduce the binding of antibody to the
platelet surface, and long-term therapy
may decreased antibody production.
(3) Enhanced vascular stability.
(4) Stimulate bone marrow to produce and
release platelet.
Usage : initial dose 1mg/kg/d.
When platelet is normal, the dose should be gradually
tapered .
·   

     2.Splenectomy

indication:

Do not respond to prednisone 3-6months

High-dose prednisone therapy >30mg/d

intolerance to prednisone
51
Cr radio-exponent in spleen area increases

Effective rate 70-90%

   3.    High-dose intravenous immunoglobulin
400mg/kg/d, 3-5days. It is highly effective in rapidly
raising the platelet count. The response rate is 90%. However,
this treatment is expensive, and the beneficial effect lasts only
1-2 weeks. Immunoglobulin treatment should be reserved for
bleeding emergencies or situations such as preparing a
severely thrombocytopenic patient for surgery.
 4.    Immunosuppressive agents
Vincristine, cyclophosphamide, azathioprine,
cyclosporine
  5.   Platelet transfusion
Disseminated Intravascular Coagulation(DIC)

General consideration a clinicopathologic

syndrom

Severe basic diseases

widespread intravascular Thromboembolism
coagulation
comsumption of platelets Bleeding Shock
and coagulation factors
secondary fibrinolysis
Pathophysiology

1.    Microthromb in small blood vessels

2.    Coagulation is abnormal
High coagulating stage
Low coagulating stage
Fibrinolysis stage
3.    Microcirculation failure
  Symptoms and Signs

Bleeding

Thromboembolism

Shock

Microangiopathic hemolysis
·  Diagnosis criteria
1.  clinical criteria
(1)   Underlying disorder that has given rise to DIC.
(2)   Two or more of following disorder
Multiple sites bleeding,
Shock,
Multiple microvascular thrombosis,
Effective anticoagulating therapy
 2.      laboratory finding criteria
(1)   PLT < 100 × 109/L or continuing decrease
(2)   Fibrinogen < 1.5 g/L or continuing decrease
or > 4g/L
(3)   3P(+) or FDP > 20 mg/L, or D-dimer ↓
(4) PT prolong or shorten > 3 s,
APTT prolong or shorten >10s
   Differential Diagnosis
Liver disease
Thrombotic thrombocytopenic purura
Treatment
1. Treatment of underlying disease
2. Anticoagulating therapy
Heparin:10000-30000u/d
Indication : (1) stage of high coagulation
(2) DIC is producing serious clinical
consequences and the underlying
cause is not rapidly reversible
Contraindication: (1) surgery
(2) severe bleeding
(3) DIC caused by venin
Monitor: APTT prolong 60%-100%
3.Replacement therapy
Platelet transfusion

Fibrinogen

Fresh plasma

Heparin must be used in combination with

replacement therapy, since heparin alone will lead
to an unacceptable increase in bleeding.

4. Antifibrinolytic therapy
The end