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Platelet disorders

Thrombopoiesis

 Platelets in bone marrow, by budding off from megakaryocytes

 Megakaryocyte and platelet production is regulated


by thrombopoietin, a hormone usually produced by the liver
and kidneys.

 Each megakaryocyte produces between 5,000 and 10,000


platelets.
 Around 1011 platelets are produced each day by an average healthy
adult.

 irregularly shaped, non-nucleated, 2–3 µm in diameter.

 Reserve platelets are stored in the spleen, and are released when
needed by sympathetically induced splenic contraction.

 lifespan of circulating platelets is 5 to 9 days.

 Old platelets are destroyed by phagocytosis in the spleen and


by Kupffer cells in the liver.
Thrombopoietin
The Role of Thrombopoietin in Platelets Production
Resting platelets

Activated platelets
General function of platelet

 The function of platelets is the maintenance of hemostasis.


 Platelets helps in blood clotting.
 Wound repair
 Platelets secrete platelet-derived growth factor (PDGF).
 Granule secretion.
 Adhesion and aggregation.
 Pro-coagulation.
 Cytokine signalling.
 Phagocytosis.
 Transport of enzyme and proteins critical to clotting.
 Formation of a platelet plug to slow blood loss.
 Contraction of a clot after it has formed, which then
reduces the size of the vessel break.
Functional
Disorders
(Qualitative)
Disorders
of
platelets
Disorders in
the number
of platelets
Platelet Functional Disorder

 Platelet functional disorder include


 Bernard - Soulier Syndrome.
 Thrombasthenia Of Glanzmann And Naegeli
(Glanzmann Thrombasthenia)
 Gray Platelet Syndrome.
 Dense Granule Deficiency Syndrome.
 Thrombotic Thrombocytopenic Purpura (TTP).
 Idiopathic Thrombocytopenic Purpura (ITP).
Bernard-Soulier syndrome
 Also known as hemorrhagiparous thrombocytic dystrophy
 It is due to deficiency of glycoprotein Ib (GPIb), the receptor for
von willebrand factor
 Lacks of GPIb cause vWF unable to bind to the glycoprotein
 finally lead to decrease in primary clot formation / primary
hemostasis
 Characterized by
 Characterized by abnormally large platelets / giant platelets
 Characterized by prolonged bleeding time, thrombocytopenia, increased
megakaryocytes, and decreased platelet survival

 Some of the symptoms:


 Purpura.
 Epistaxis.
 Menorrhagia.
 Gingival and gastrointestinal bleeding.
THROMBASTHENIA
OF GLANZMANN AND NAEGELI
 extremely rare coagulopathy

 can be inherited in an autosomal recessive manner or acquired as an


autoimmune disorder

 due to deficiency in glycoprotein IIb/IIIa (GpIIb/IIIa)

 glycoprotein IIb/IIIa (GpIIb/IIIa) is receptor for fibrinogen.

 When glycoprotein IIb/IIIa (GpIIb/IIIa) receptor is dysfunction, fibrinogen


cannot bind to the platelets.

 As a result, no fibrinogen bridging of platelets to other platelets occur

 In other words, primary hemostasis inhibited and prevent platelets


aggregation

 Bleeding time is significantly prolonged


THROMBASTHENIA
OF GLANZMANN AND NAEGELI
 Symptoms includes:

 Increased mucosal bleeding.

 Epistaxis.

 Menorrhagia.

 Increased bleeding post-operatively.

 The bleeding tendency is variable but may be severe.

 Platelet numbers and morphology are normal.

 Platelet aggregation is normal with ristocetin, but impaired with


other agonists such as ADP, thrombin, collagen or epinephrine
Gp Ib receptor on platelets
Gp IIb-IIIa complex on platelet surface
GRAY PLATELET SYNDROME
 Gray platelet syndrome (GPS) is a rare inherited bleeding disorder.
 The abnormal alpha-granules appear grey on blood films stained by
the May-Grünwald-Giesma stain - hence, the syndrome's name.
 It caused by the inability of platelets to store alpha-granule proteins.
 The platelets' haemostatic proteins are not released at the site of
vascular injury.
 Thus slows aggregation and vessel repair
 And contribute to the bleeding tendency.
 Symptoms may include:
 platelets that have a gray appearance
 severe thrombocytopenia
 myelofibrosis
 splenomegaly
DENSE GRANULE DEFICIENCY
SYNDROME
 it is a bleeding disorder caused by a deficiency in dense
granules in the platelets

 Dense granules release chemicals in the clotting process and


help platelets stick together to form clots.

 Lacks of dense granules in platelets means lacks of storage sites


for substances for clotting.

 Therefore no chemicals which facilitates in the clotting process


will be released.

 Finally leads to slow platelet activation, and prolonged bleding.


Bleeding time
Disorders in the number
of platelets
Thrombocytopenia
• Thrombocytopenia  a platelet count of less than 150,000/mm3
(<150,000/mm3)
• N.B. With normal platelet function, thrombocytopenia is rarely the
cause of bleeding unless the count is less than 50,000/mm3.
• Thrombocytopenia should always be confirmed by examination of a
peripheral smear.
• It can be caused by:
– decreased platelet production
– increased destruction
– Sequestration (DISORDERS OF DISTRIBUTION of platelets)
– or a combination of these causes.
THROMBOTIC THROMBOCYTOPENIC
PURPURA
 A blood disorder that causes blood clots to form in small blood vessels
around the body, and leads to a low platelet count.
 The two main types of TTP are inherited and acquired.
 In inherited TTP, the ADAMTS13 gene is faulty and doesn't prompt the
body to make a normal ADAMTS13 enzyme. As a result, enzyme activity is
lacking or changed.
 Acquired TTP is the more common type of the disorder. The ADAMTS13
gene isn't faulty. Instead, the body makes antibodies (proteins) that block
the activity of the ADAMTS13 enzyme.
 A lack of activity in the ADAMTS13 enzyme causes TTP.
 The ADAMTS13 gene controls the enzyme, which is involved in blood
clotting.
 The enzyme breaks up a large protein called von Willebrand factor that
clumps together with platelets to form blood clots.
IDIOPATHIC THROMBOCYTOPENIC
PURPURA
 Also known as immune thrombocytopenic purpura, is classified as
an autoimmune disease.

 The term “idiopathic” indicates that the disease is of an unknown


cause or origin: in other words, modern medicine has not yet
figured out what it is.

 And the word “purpura” comes from a description of the bruise-


colored skin of someone afflicted with the disease: the purple
color caused by blood that leaked under the skin.
Estimated proportion of different causes of ITP
IDIOPATHIC THROMBOCYTOPENIC PURPURA
 Idiopathic thrombocytopenic purpura is a bleeding disorder in which
the immune system destroys platelets
 Persons with the disease have too few platelets in the blood
 The two types of ITP are acute (temporary or short-term) and chronic
(long-lasting).
 Acute ITP generally lasts less than 6 months. It mainly occurs in
children—both boys and girls—and is the most common type of ITP.
Acute ITP often occurs after a viral infection.
 Chronic ITP lasts 6 months or longer and mostly affects adults.
However, some teenagers and children do get this type of ITP.
Chronic ITP affects women two to three times more often than men.
 Symptoms:
 Abnormally heavy menstruation.
 Bleeding into the skin causes a characteristic skin rash that looks like
pinpoint red spots. (petechial rash)
 Easy bruising.
 Nosebleed or bleeding in the mouth
Clinical Presentation
When to Treat
First-line treatment options for adult ITP patients
Thrombocytosis
Increased amount of platelets in blood
Thrombocytosis is almost always due to:
- Iron deficiency
- Inflammation
- Cancer
- Infection (reactive thrombocytosis)
- An underlying myeloproliferative process [essential
thrombocythemia or polycythemia vera or, rarely,
the 5q-myelodysplastic process.

Medications that can cause reactive thrombocytosis include:


- Epinephrine (Adrenalin Chloride, EpiPen)
- Tretinoin
- Vincristine

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