Fourth Stage

General Medicine

‫ علي سلمان جاسم‬.‫د‬.‫م‬

Lecture 3
 Lec3 Dr.Ali Salman Almamoori

Hemostasis
The process of stopping or arresting bleeding or keeping the
blood within the damaged blood vessel, particularly by
physiological means such as vasoconstriction and coagulation
or by surgical means.
The mechanism of hemostasis can divide into four stages .
1) Constriction of the blood vessel.
2) Formation of a temporary “platelet plug.
3) Activation of the coagulation cascade.
4) Formation of “fibrin plug” or the final clot

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Lec3 Dr.Ali Salman Almamoori

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 Lec3 Dr.Ali Salman Almamoori

Bleeding Disorder.

Idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is immune-mediated with
involvement of autoantibodies, most often directed against the platelet
membrane glycoprotein IIb/IIIa, which sensitise the platelet, resulting in
premature removal from the circulation by cells of the reticulo-
endothelial system.
Clinical features and investigations
The presentation depends on the degree of thrombocytopenia.
Spontaneous bleeding typically occurs only when the platelet count is
below 20×109/L.
At higher counts, the patient may complain of easy bruising or sometimes
epistaxis or menorrhagia.
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 Lec3 Dr.Ali Salman Almamoori

Management: Many patients with stable compensated ITP and a platelet

count of more than 30×109/L do not require treatment to raise the
platelet count, except at times of increased bleeding risk, such as surgery
and biopsy.

First-line therapy for patients with spontaneous bleeding is with high

doses of glucocorticoids, either prednisolone (1 mg/kg daily) or
dexamethasone (40 mg daily for 4 days), to suppress antibody production
and inhibit phagocytosis of sensitized platelets by reticulo-endothelial
cells. Administration of intravenous immunoglobulin can raise the platelet
count by blocking antibody receptors on reticulo-endothelial cells, and is
combined with glucocorticoid therapy if there is severe haemostatic
failure, especially with evidence of significant mucosal bleeding or a slow
response to glucocorticoids alone. Persistent or potentially life-threatening
bleeding should be treated with platelet transfusion in addition to the
other therapies.

Coagulation Disorder

Von Willebrand disease is the most common inherited bleeding disorder.

Hemophilia A and B are the most common single coagulation factor
deficiencies but inherited deficiencies of all the other coagulation factors
are seen. Acquired disorders may be due to under-production (e.g. in liver
failure), increased consumption (e.g. in DIC) or inhibition of function of
coagulation factors (such as heparin therapy or immune inhibitors of
coagulation, e.g. acquired haemophilia A).

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 Lec3 Dr.Ali Salman Almamoori

haemophilia A

Factor VIII deficiency resulting in haemophilia A affects 1/10 000

individuals. It is the most common congenital coagulation factor
deficiency. As the factor VIII gene is on the X chromosome, haemophilia A
is a sex-linked disorder. Thus all daughters of a patient with haemophilia
are obligate carriers and they, in turn, have a 1 in 4 chance of each
pregnancy resulting in the birth of an affected male baby, a normal male
baby, a carrier female or a normal female. Antenatal diagnosis by
chorionic villous sampling is possible in families with a known mutation.

Management

The key to the management of severe haemophilia A in more affluent

countries is prophylactic coagulation factor replacement. The aim of this
treatment is to maintain trough levels of factor VIII (or IX in the case of
haemophilia B) above 0.02 U/mL. Doing this substantially reduces the
number of bleeding episodes for men with severe haemophilia and so
reduces the rate of deterioration of joints, which is the major long-term
morbidity.
The vasopressin receptor agonist desmopressin raises the vWF and factor
VIII levels 3–4-fold, which is useful in arresting bleeding in patients with
mild or moderate haemophilia A.

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 Lec3 Dr.Ali Salman Almamoori

Haemophilia B (Christmas disease)

Aberrations of the factor IX gene, which is also present on the X chromosome,

result in a reduction of the plasma factor IX level, giving rise to hemophilia B.
This disorder is clinically indistinguishable from hemophilia A but is less
common. The frequency of bleeding episodes is related to the severity of the
deficiency of the plasma factor IX level. Treatment is with a factor IX
concentrate, used in much the same way as factor VIII for haemophilia A.

ASSESSMENT OF COAGULATION SYSTEM

Bleeding time: Time for a small puncture wound in the skin made by
standard technique to stop bleeding. • Time varies from 1 to 8 min. A time
within this range implies:
■ adequate platelet count
■ normal platelet function
■ normal vascular response to injury.
• A prolonged bleeding time implies:
■ thrombocytopenia
■ platelet defects
■ failure of vascular contraction.
Whole-blood clotting time • Blood clots in glass tube in 5–15 min.
• A clotting time within this range requires:
■ integrity of the intrinsic system
■ adequate final common pathway
■ normal platelet function. 6
 Lec3 Dr.Ali Salman Almamoori

Prothrombin times (PT)

• Tests the integrity of the
extrinsic pathway and final common pathway.
• Deficiencies of Factors I, II, V, VII and X will be detected.
Activated partial thromboplastin time (APTT)
• Tests the intrinsic system, i.e. all factors except Factor VII.

Thrombin Time (TT)

• Increased if there is an inadequate concentration of fibrinogen.
• Prolonged by heparin and presence of fibrin degradation products.

Fibrin degradation products (FDPs)

• Products released from fibrinogen and fibrin by plasmin.
• Increased in disseminated intravascular coagulation (DIC).

T Fibrin degradation products (FDPs)

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 Lec3 Dr.Ali Salman Almamoori

Assessment of the different pathways involved in coagulation may be

made with two simple tests:
• APTT for intrinsic system
• PT for extrinsic system.

Dental Issue
if patient has history of a bleeding disorder it is fine for you to attend
your own dentist for routine care such as check-ups and fillings. For more
invasive treatment such as tooth extractions, gum surgery or some
deeper injections (nerve blocks . dentist must refer the patient physician
to control the case.