Rayneilda Eleana Siew

MLT U29
UNIT MAKMAL PATOLOGI
HOSPITAL NUKLEUS WP LABUAN
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Classified into;
1.
2.

Quantitative defects (decrease or increase in number)

Qualitative defects (Normal in count but abnormal in
function)

Characteristic clinical feature;

bleeding either into the subcutaneous tissues or from
the mucosa.
Classical signs of hemorrhage symptoms
Purpura
gingival bleeding
Nasal bleeding
Melena
Hematuria, menorrhagia, etc.

i.
ii.
iii.
iv.
v.

1.

THROMBOCYTOPENIA (DECREASED OF
PLATELETS)

Decrease in peripheral blood count when;

i.

Reduced production

ii.

Excessive destruction

iii.

Excessive consumption

Classified into;
a.

Congenital

b.

Acquired

a.

Congenital thrombocytopenia

Neonatal thrombocytopenia - an immune reaction;

transplacental passes of maternal antibodies.

Kasabach-Merritt Syndrome massive intravascular

clotting in large hematoma.

Wiskott-Aldrich syndrome autosomal recessive, small

platelets

Bernard-soulier syndrome autosomal recessive disorder,

giant platelets

May-Hegglin anomaly autosomal dominant, giant platelets

and basophilic inclusions (dohle bodies) in granulocytes.

b. Acquired thrombocytopenia
i.

Idiopathic (autoimmune) thrombocytopenic purpura (ITP,

AITP)

ii.

Systemic Lupus Erythematosus (SLE)

iii.

Consumption thrombocytopenias

Includes disseminated intravascular coagulation (DIC),

microangiophatic hemolytic anemia, thrombotic
thrombocytopenic purpura (TTP).

iv.

Suppression of bone marrow megakaryocytes

Aplastic anemia, infiltration of bone marrow, etc.

2. INCREASED OF PLATELETS
a.

Thrombocytosis

Platelet counts increased to > 500 X 109/L

Is secondary to acute or chronic bleeding, iron

deficiency anemia, malignant lymphoma, cancer ad
other condition.

b.

Thrombocythemia

Is a chronic myeloproliferative disorder, which

platelet count is persistently elevated to 1000
1,500 X 109/L

Characterized by bleeding tendency associated with

thromboembolic.

May congenital or acquired

Acquired form more common and occurs

secondary to a variety diseases and also to
administration of medicine.

Platelet count usually normal, bleeding

occurs because of defects in platelets
function.

DISORDER OF MEMBRANE GLYCOPROTEIN

i.

Benard-Soulier syndrome (BSS)

Autosomal recessive inheritance

Due to lack of the GPIb-IX complex of the platelet

membrane glycoprotein.

Defect in platelet adhesion.

Bleeding Time prolonged, decreased platelets

count with giant platelets.

ii.

Glanzmanns thrombasthenia

An abnormality of platelets.

Rare coagulopathy,in which the platelets contain

defective or low levels of Glycoprotein
IIb/IIa(GPIIb/IIa),which is a receptor for Fibrinogen
(FI)

No aggregation occurs.

VON WILLEBRAND DISEASE (vWD)

Hemorrhagic tendency due to decreased(quantitative) or

qualitative abnormalities in von Willebrand Factor (vWF).

Divided into three types;

i.

Type I : classical form,60-80% of all vWD cases

(quantitative defect-heterozegous for the defective gene).

ii.

Type II : 20-30% of cases ,variant form (qualitative

abnormalities with defect in large multimere).

iii.

Type III severe form; platelet bind to large multimere of

vWF excessively resulting in reduction of this protein.
Primarily defect is the platelets not in vWF.

In various diseases

Much greater than the inherited syndrome

Produces multiple functional abnormalities.

Complete recovery expected following successful

treatment of underlying disease.

a)

Chronic renal failure (Uremia)

Decreased platelet count

Decreased coagulation factor

Uremic drugs inhibit of platelet secretion

b.

Dysproteinemia

Multiple myeloma and macroglobulinemia

Platelet function impaired due to coating of

the platelet membrane by paraprotein

c.

Myeloproliferative disorder

Defect in platelet aggregation

d.

Drug induced qualitative platelet defect

Platelets
Bleeding
Clot

time Duke method or Ivy Method

retraction

Platelet

factor assays

BSS

Glanzmann Thrombasthenia

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May-Hegglin Anomaly
Wiskott-Aldrich Syndrome
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1.

ADP aggregation test

a.

Instrument and reagent;

Sample from vein with 3.2 % citrate as anti coagulant (0.5 ml +

4.5 ml blood)

Small plastic test tube

ADP solution; 10 mol/L ADP

Microscope

b.

Procedure;

i.

Spin the blood at 1000 rpm for 10 min and separate the platelet
rich plasma (PRP)

ii.

Add 0.2 ml PRP with 0.1 ml ADP solution and shake gently at
room temperature.

iii.

Observe under microscope for the aggregation formation.