Congenital: hemophilia A – Factor VIII (8) _
Intrinsic pathway – Hemophilia B
–Christmas disease -Factor IX(9) _ Intrinsic
pathway – Von willebrand diseasen
Platelets disorders
Acquired: Aspirin- uremia
(RF)-MPD Inherited : von
Willebrand Disease
Function:Disorders of
platelet function
Number:
Thrombocytopenia (ITP)
Investigations
igG antibodies produced by B-cells
that were used to help in infection
against a certain pathogen
mononuclear macrophages in the
red pulp destroy
immunoglobulin-coated
platelets(acute ITP – younger kids),
While in chronic its just idiopathic
Differential Diagnosis
Since ITP is a diagnosis of exlusion, it is
important to rule out other causes of
low platelets like: 1. SLE 2.Infections 3.
Common variable immunodeficiency 4.
Vaccinations 5. Medications (anti
epilepsy) 6. Hodgkin lymphoma
Signs & Symptoms
Purpura
Non-thrombocytopenic
Purpura
Senile Purpura
Allergic Purpura
Thrombocytopenic
Purpura
Thrombotic Thrombocytopenic Purpura
Idiopathic Thrombocytopenic Purpura
Purpura is a skin rash usually
characterized by small, purplish-red
spots on the skin. Size: 0.3 cm- 1 cm
Bleeding from the
gums or nose
Blood in urine or stools
Petechiae
Management
Blood or platelet transfusions.
replace lost blood
Medications
Corticosteroids
such
as
prednisone
Immune globulin.
Other drugs
Rituximab
If corticosteroids
don't help
Surgery
Splenectomy
This quickly eliminates the main source of
platelet destruction in the body and improves
the platelet count but permanently increases
susceptibility to infection.
Vaccinations
Pneumococcus vaccine (Pneumovax)
Meningococcal vaccine
Haemophilus B vaccine
Complications
Blood clot in the vein that carries
blood to the liver - Hernia at the
incision site - Infection at the
incision site - Inflammation of the
pancreas (pancreatitis) - Lung
collapse Injury to the pancreas,
stomach, and colon
Prognosis
Adults
The symptoms may come and
go. After splenectomy 70%
percent of the platelets are
restored. (CHRONIC)
Children
symptoms resolving in
less than 6 months
(couple weeks)...doesn’t
recur....Recovery 75-80
percent within 3-6
months (ACUTE ITP)
Overall The disease is not life threatening even for people
with severe cases. It doesn’t seem to decrease the life
span for it’s affected patients.
Relapse
Differs in patients, Platelets may undergo
protein degradation by antigen presenting cells
(APC) followed by antigen presentation to T cells.
The chronic aspect of the disease may be the
result of a B and T cell memory response.
Epidemiology
Etiology
immune system
mistakenly attacking
and destroying platelets
Vascular bleeding disorders
Congenital defects of
the vessel wall Ehlers-
Danlos syndrome