Zusammenfassung der Ressource
SANA WITH RASH AND EASY BRUISING
- Coagulation Cascasde
- Bleeding Disorders
- Coagulation disorders
- Acquired: Liver disease
- 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
- Senile purpura
- Scurvy vitamin C
deficiency
- Septic Vasculitis
Infections &
Henoch-Schonlein
Purpura