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Platelets disorders seminar

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mohd rasmi

on 22 December 2014

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Transcript of Platelets disorders seminar

Heme & Immune Block
4th Seminar
Disorders of Platelets
Name :Mohammed Rasmi Almutairi
ID : 331100222

Objectives :
1- Define thrombocytopenia, thromboasthenia and thrombocytosis

2- List causes of thrombocytopenia and thromboasthenia

3- Describe the pathogenesis of thrombocytopenia
Firstly
What is the main function of platelets ?
Prevent bleeding " loss of blood"
So
Platelet count: Normally, it is
150.000 - 450.000 mm3.

Qs : If there are excessive platelets in the blood , will lead to ?
1- Stroke
2- Heart attack
3- clot in blood vessels
4- all of them
1- Stroke
2- Heart attack
3- clot in blood vessels
4- all of them

Thrombocytosis
It is a disorder in which the body produces too many platelets (thrombocytes), more than the normal range count
Secondary thrombocytosis
Primary
thrombocytosis
If there is :
inflammation , infection,some surgery & iron deficiency
abnormal cells "neoplastic" in the bone marrow cause an increase in platelets
Qs : Thrombocytosis which caused by Splenectomy is classified into which types of the following ?
1- Primary thrombocytosis
2- Essential thrombocythemia
3- Secondary thrombocytosis
4- Essential thrombocytosis
1- Primary thrombocytosis
2- Essential thrombocythemia
3- Secondary thrombocytosis
4- Essential thrombocytosis
Thrombasthenia
- Introduction
The patient suspected to show skin & mucosal haemorrhage despite :
a normal platelet count & normal level of VWF
Thrombasthenia
" Glanzmann's Disease"
2- Which one of the following is the best statement about thrombasthenia :
A. High decreasing in platelet count
B. Normal platelet count with no clinical features
C. Normal platelet count with severe bleeding
D. Normal platelet count with failure of platelet
aggregation
A. High decreasing in platelet count
B. Normal platelet count with no clinical features
C. Normal platelet count with severe bleeding
D. Normal platelet count with failure of platelet
aggregation
2- Which of the following is the type of thrombasthenia "hereditary" :
A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive
A. Autosomal dominant
B. Autosomal recessive
C. X-linked dominant
D. X-linked recessive
Thrombocytopenia
* Introduction :
Defined as a blood platelet count below 150000mm3
But
This condition, which usually affects children and young adults, may be triggered by a viral infection such as measles
ITP
A significant feature of this disease is the
presence of
Purpura
, which are haemorrhage into the skin ( purpura - petechiae)
Acute
Chronic
Typically appears in
children
of either gender after a
viral illness
and is likely caused by virus-induced changes in platelet antigens that elicit
autoantibodies
. Complement bound at the surface causes platelets to be lysed in the blood or phagocytosed and destroyed by splenic and hepatic macrophages .

Occurs mainly in

adults

and may be associated with

collagen vascular diseases

( e.g. systemic lupus erythematosus SLE ) or

a malignant lymphoproliferative disease

, especially
Chronic

lymphocytic leukemia

. It is also common in people infected with

HIV

Spontaneous capillary bleeding , when platelets below 20000mm3 & increase
bleeding time when it below 90000mm3
Qs : Which of the following is the best significant feature of ITP
1- Fissuring of the skin
2- Ecchymosis
3- Purpura
4- Coloration of the skin
1- Fissuring of the skin
2- Ecchymosis
3- Purpura
4- Coloration of the skin
Drug-Induced Thrombocytopenia
" Heparin"
it cause immune-mediated platelet destruction.
it forms a complex with a platelet-related protein that elicits antibody production.
By IgG antibodies against PF4/heparin complex
Exacerbate thrombosis
Qs : the mechanism of heparin- induced thrombocytopenia, is classified as :
1- Increase production of platelets
2- Destruction of platelets
3- Dilutional
4- Sequestration
1- Increase production of platelets
2- Destruction of platelets
3- Dilutional
4- Sequestration
TTP
* Deficiency of
metalloprotease
, that normally degrade very-high-molecular-weight multimer vWF , that lead to accumlation of vWFs
in plasma and promote aggregation
Hemolytic-Uremic syndrome
* Due to damage of endothelium as
a result of certain causes
e.g. infection by E.coli
Qs : Which of the following is the cause of HUS
1- Drug like heparin
2- Bacterial infection e.g. E.coli
3- Antibodies against platelets
4- Viral infection e.g. HSV
1- Drug like heparin
2- Bacterial infection e.g. E.coli
3- Antibodies against platelets
4- Viral infection e.g. HSV
Summary
Thrombocytosis:
Increase platelets count
Thrombasthenia:
functional disorder of platelets
" deficiency of GP IIb/IIIa"


Causes : 1- hereditary 2-Acquired
Thrombocytopenia:
Decrease platelets count.
Causes :
1- Decrease production
2- Decrease platelets life span
3- Sequestration
4- Dilutional
1- The condition when there is a deficiency of GPIIb/IIIa is called :
A- Bernard-Soulier Syndrome
B- Immune Thrombocytopenia Purpura
C- Glanzmann's Disease
D- Hemolytic-Uremic Syndrome
2- Which one of the following drugs may cause impair platelets production:
A- Heparin
B- Quinine
C- Ethanol
D- Quinidine
3- Neoplastic cells of bone marrow "stem cell" lead to which type of thrombocytosis :
A- Primary
B- Secondary
C- Tertiary
D- Quaternary
4- TTP, is a condition of deficiency of which of the following enzyme :
A- G6PD
B- Protease
C- Oxidase
D- Metalloprotease
5- Mechanism of heparin-induced thrombocytopenia is to produce which of the following antibodies :
A- IgA
B- IgM
C- IgG
D- IgD
References :
Qs : In thrombocytopenia , bleeding time will increase than normal when the platelet count :
A- 4500000 mm3
B- 80000 mm3
C- 150000 mm3
D- 220000 mm3
A- 4500000 mm3
B- 80000 mm3
C- 150000 mm3
D- 220000 mm3
- Introduction
The Function defects , may be because :
Abnormal size
deficiencies of platelet : adhesion, aggregation, and secretion.
deficiencies in some factors
Def.
GT is a rare autosomal recessive disorder whereby the quantity or quality of platelet membrane glycoprotein
(GP) IIb-IIIa
is abnormal, preventing the aggregation of platelets and subsequent clot formation.
Causes Glanzmann’s Disease ?
The genes for
glycoprotein IIB/IIIA
are carried on
chromosome 17
of the
DNA
. Then there are defects in these genes, it can lead to Glanzmann’s.

The condition is
autosomal recessive
, meaning that both of the parents must carry the defective gene or genes for Glanzmann’s in order for you to inherit the disease.
Full transcript