Transcript Thrombocytopenia

Disorder of hemostasis. Haemophylia.
Trombocytopenias. Willebrand’s disease.
Thrombohemorrhagic syndromes. Etiology.
Pathogenesis. Diagnostics. Clinical pattern.
Principles of treatment. Typical changes in oral
cavity in agranulocytosis and leukaemias. The
role of a dentist in early diagnostics and
prophylaxis
N.Z. Yarema, ass.proff.
Hemorrhagic diathesis – group of
diseases, which characterized by
bleeding sickness due to
hemostasis disorders
Hemostasis or haemostasis is a
complex process which causes the
bleeding process to stop.
The main coagulogram indexes
1
Index
Hypo
coagulation
Normo
coagulation
Hyper
coagulation
Bleeding time (by LeeWhite methods), min
>5
5-3
<3
Platelets number
<180
180-320
>320
Platelet adhesiveness
<23
23-44
>45
Time of plasma
recalcification, sec
>120
120-60
<60
Heparin tolerance test,
min
>11
11-8
<8
Prothrombin index, %
<80
80-100
>100
U-factor
<80
80-100
>100
Procorventin (VII factor),
%
<80
80-100
>100
Fibrinogen, g/l
<2
2-4
>4
Hemorrhagic diathesis
 Bleeding diathesis is an unusual
susceptibility to bleeding (hemorrhage)
due to a defect in the system of
coagulation. Several types are
distinguished, ranging from mild to lethal.
Hemostatic mechanism
 The classic hemostatic mechanism
include:
 Platelets adhesion
 Coagulation factor
 Vascular response
The main bleeding sickness types
 Angiomatosic (hemorrhage due to vascular dysplasia,
teleangiectasia; the main clinical criteria is relapsing
bleeding without hemorrhage in skin, subcutaneous and
other tissue; nose bleeding are most often, dangerous and
massive)
The main bleeding sickness types
 Petechia-ecchymosis (is usually localized to superficial sites
such as the skin and mucous membranes, wich often combined with
menorrhagia, nose bleeding, gumms bleeding; rare – with
gastrointestinal bleeding or brain hemorrhage; it is small, painless,
provoked by simple action like skin cleaning, measure of blood pressure
etc; pinch test is positive)
The main bleeding sickness types
 Hematomic
(massive, deep, painful; bleeding may occur anywhere.
The most common sites of bleeding are into joints (knees, ankles, elbows),
into muscles, from the gastrointestinal tract, cause of the bleeding can be
intramuscular injection; characterized by early postoperative &
posttraumatic bleeding)
The main bleeding sickness types
 Mixed (Petechial & Hematomic)
(combined features of both types,
but there are some difference: in contrast to hematomic - bleeding are into joints
very rare, mostly it is located in subcutaneous, retroperitoneal, mesenteric,
subserous intestinal layer or into internal organ);in contrast to petechial-ecchymosic
bleeding - hemorrhagic syndrome characterized by large bruise)
The main bleeding sickness types
 Vasculatic (hemorrhage due to inflammatory changes of
small vessels, the main cause are immune disorders or
infectious agent)
Classification of Hemorrhagic diathesis
o Thrombocytopenia (decrease in the number of
platelets)
o
o
o
o
- Idiopathic thrombocytopenic purpura (ITP)
- Thrombotic thrombocytopenic purpura (TTP)
- Heparin-induced thrombocytopenia (HIT)
- Hemolytic-Uremic Syndrome
 Coagulopathies (disorders of bleeding and
coagulation)
Disseminated intravascular coagulation
o
o
o
o
Hemophilia
Hemophilia A
Hemophilia B (Christmas disease)
Hemophilia C
 Von Willebrand disease
 Vasculitis
THE CLOTTING MECHANISM
INTRINSIC
Collagen
XII
XI
EXTRINSIC
Tissue
Thromboplastin
VII
IX
VIII
X
FIBRINOGE
(I)
N
V
PROTHROMBIN
(II)
THROMBIN
(III)
FIBRIN
Bleeding
disorders
Vascular
abnormalities
***
Platelet disorders
Clotting factor
abnormalities
DIC
Bleeding disorders
Platelet disorders
↓production
↑destruction
Primary/Idiopathic
ITP
Acute/Chronic
Sequestration
Hypersplenism
Secondary
Drugs, HIV
Thrombocytopenia
 Thrombocytopenia (or -paenia, or thrombopenia in short) is the




presence of relatively few platelets in blood.
Generally speaking a normal platelet count ranges from 180,000 and
320,000 per mm3.
Signs and symptoms
Often, low platelet levels do not lead to clinical problems; rather, they are
picked up on a routine full blood count. Occasionally, there may be
bruising, nosebleeds and/or bleeding gums.
It is vital that a full medical history is elicited, to ensure the low platelet
count is not due to a secondary process. It is also important to ensure
that the other blood cell types red blood cells, and white blood cells, are
not also suppressed.
Thrombocytopenia
Diagnostic
criteria
Petechialecchymosic
1. Deacrease
of platels
Platelet disorders
 Thrombocytopenia =Reduced platelet number
 Causes




Decreased production of platelets
 vitamin B12 or folic acid deficiency
Decreased platelet survival
 Immunologic or Nonimmunologic etiology
Sequestration- Hypersplenism
 ameliorated by splenectomy
Dilutional
 Massive transfusions
Thrombotic Microangiopathies
1.
2.
Thrombotic thrombocytopenic Purpura (TTP)
Hemolytic-Uremic syndrome (HUS)
Petechia
 small red or purple
spot on the body,
caused by a minor
hemorrhage (broken
capillary blood
vessels).
Bleeding
disorders
Vascular
abnormalities
Platelet disorders
Clotting factor
abnormalities
DIC
Clotting factor abnormalities
 Congenital disorders
 Von Willebrand disease –MC with minimal bleeding
 Factor VIII Deficiency - Hemophilia A or Classic Type
 Factor IX Deficiency – Hemophilia B
 Acquired disorders
 Vit. K deficiency =Due to deficient carboxylation of factors II,
VII, IX &X
 Oral anti-coagulants
 Coumarin derivatives = warfarin – inhibit Vit. K factors
 Liver diseases ↓ synthesis of factors
Hemophilia
Diagnostic
criteria
Hematomic
bleeding sickness
types
Arhtropathy
von Willebrand's Disease
Essentials of Diagnosis
 Family history with autosomal dominant pattern of inheritance.
 Prolonged bleeding time, either at baseline or after challenge with
aspirin.
 Reduced levels of factor VIII antigen or ristocetin cofactor.
 Reduced levels of factor VIII coagulant activity in some patients.
Symptoms and Signs
 von Willebrand's disease is a common disorder affecting both men
and women. Most cases are mild. Most bleeding is mucosal (epistaxis,
gingival bleeding, menorrhagia), but gastrointestinal bleeding may
occur. In most cases, incisional bleeding occurs after surgery or
dental extractions. von Willebrand's disease is rarely as severe as
hemophilia, and spontaneous hemarthroses do not occur (except in
the rare type III). The bleeding tendency is exacerbated by aspirin.
Characteristically, bleeding decreases during pregnancy or estrogen
use.
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Haemophilia
А
factor VIII
deficiency
В
factor IX
deficiency
С
factor XI
deficiency
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Forms
 Haemophilia A - factor VIII deficiency, "classic haemophilia"
(X-linked)
 Haemophilia B - factor IX deficiency, "Christmas disease" (Xlinked)
 Haemophilia C - factor XI deficiency (Ashkenazi Jews,
autosomal recessive)
 The unrelated type 1 and type 2 von Willebrand disease (vWD)
Classification (F VIII C
level)
2
1
F
VIII C
level 5-15
%
Mild form
F
VIII C
level 1-5
%
Moderate
form
3
F
VIII C
level less
than 1 %
.
Severe form
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Hemophilia
A Treatment (cryoprecipitate)
1
Mild
15-20 UN/kg
2
3
Moderate
Severe
.
35-40 UN/kg
70 UN/kg
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Hemophilia
B Treatment (cryopLASMA)
1
Mild
2
Moderate
3
Severe
.
15-20 ml/kg 35-40 ml/kg 70ml/kg
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Bleeding
disorders
Vascular
abnormalities
Platelet disorders
Clotting factor
abnormalities
DIC
Bleeding
disorders
Vascular
abnormalities
Platelet disorders
Clotting factor
abnormalities
DIC
The main bleeding types
Petechial-ecchymosis
Hematoma
Mixed (Petechia & Hematoma)
Vasculatic
Angiomatosic
Screening tests for bleeding disorders
Test
Abnormality detected
Blood count and film
Anaemia, leukaemia, disseminated
intravascular coagulation
Platelet count
Thrombocytopenia
Activated partial thromboplastin time
Deficiency of all coagulation factors
except VII, especially follows VIII and IX;
heparin
Prothrombin time
Deficiency of factors I, II, V, VII, and X;
warfarin
Thrombin time or fibrinogen
Hypofibrinogenaemia or
dysfibrinogenaemia; heparin; fibrin
degradation products
Test of platelet-vessel wall interaction
Bleeding time
Thank you for attention!