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Platelet disorder
Thrombocytopenia Thrombasthenia
Blood vessel
Vasoconstriction Vascular
injury
phase
Platelet Consolidation
Platelet Platelet
aggregation Platelet plug
adhesion formation phase
Prothrombin
Tissue Plasma
thrompoplastin factors
Thrombin Plasma
phase
fibrinogen Fibrin
Concept of hemostasis
Vessel injury Vasoconstriction
Thrombin
Platelet plug
DD
Steroid Purpura: of Racjhitic rosaries
Due to prolonged
use of
- Rickets: corticosteroids
enlarged either systemic
costochondral juction or
topical → thining of wall of
- Marsmus: blood vessel.
rounded , normal sized
May be in cushing syndrome (Endogenous ↑ ).
costochondral juncion, not tender
Fat Embolism:-Achondroplasia: angulation , angle downword (V)
As in fracture-Scurvey: tender
of long Bones.
I- Vascular Purpura
Traumatic Asphyxia: as:
Severe chronic cough e.g. Whooping cough.
Epileptic fits.
Severe infections:
Subacute bacterial endocarditis, Typhoid fever, menigococcal
septicemia → Due to vasculitis.
Uremia.
Diabetes Mellitus.
Drugs: Penicillin, sulfa, INH, mercurials….etc.
Due to hypersensitivity to drug
Purpura Facticia: “Self inflected”.
Usually in the areas within reach.
More common in females.
I- Vascular Purpura
B) Congenital:
Congenital Amyloidosis.
Heriditary telengiactasia (Rendu-Osler-Weber disease).
Autosomal dominant disease.
Thin walled dilatations in the wall of blood capillaries. These
dilatations lack the muscle layer so, if injured they can not
contract like other normal BI.v. → severe bleeding.
Ehlers- Danlos Syndrome:
Autosomal dominant disease characterised by:
↑ Skin Elasticity.
↑ Range of movement of joints ( Hyper extensibility of joints).
↑ Fragility of blood vessels.
Anaphylactoid Purpura
Definition
Allergic disorder similar to
Rheumatic fever in which the
allergic reaction occurs in wall of
blood vessels.
Etiology
May be 2ry to infection with group A B
hemolytic streptococci.
Or
Other infections.
Insect bites.
Food or drug allergy.
Clinical Picture
It can affect one or more of 4 systems:-
Skin: 100%
Youmaculopapular
Urticarial can’tMedical
Diagnose Causes
rash anaphylactoid
present of Acute Abdomen:-
manily on lower limbs,
-Diabetic Buttocks & Extensor surface of
Ketoacidosis
upperpurpura
limbs. without skin Manifestations
-Sickle cell crisis
Ecchymosis & purpuric
-Rh fever(eruption.
activity)
Angioneurotic edema is common.
-Anaphylactoid
Joints: 75%
purpura ( mesentric vasculitis)
-Basal pleurisy
Migratory polyarthritis or arthralgia similar to that of
rheumatic fever but-Uraemia
the joints are not tender.
-Familial
Gastro-intestinal Mediterranean fever
tract: 50%
Severe abdominal-Porphyria
pain.
Diarrhea. Mesentric Vasculitis
-Inferior wall infarction DD from
Bloody stools.
Renal: 25% Appendicitis
It may be in the form of asymptomatic proteinuria and
hematuria or nephrotic syndrome.
Clinical Picture
Clinical Picture
Complications
Intussusception.
Subarachinoid or cerebral hemorrhage.
Investigations
All investigations of bleeding tendency
are normal except Hiss test may be +ve.
Diagnosis depends on clinical
So, diagnosis depends on clinical
manifestationsmanifestations
with:
1- Investigations for streptococcal infection:
* ESR * ASO Titer
2- Urine analysis:
Technique
* To detect of Hiss
renal affection Test?
(worst prognosis) because
50% of cases → chronic renal failure.
3- Stool analysis: for occult blood.
4- Serum IgA: may be elevated.
Treatment
This disease is self limited but recurrence can
occur at least within 6 months.
Pencillin:
If the condition is 2ry to streptococcal infection.
And if not try to detect the cause and avoid it as drugs or
insect bites.
For Arthritis:
Acetyl salicylic acid as for rheumatic arthritis.
For GIT manifestations:
Corticosteroids: Prednisone 1-2mg/kg/day oral.
For renal Affection:
Immuno suppressive drugs as: Endoxan, and
azothioprine.
II- Platelet Disorder
Platelets are essential for Haemostasis by:
Their number.
Their function:
Platelet aggregation .
Platelet adhesiveness.
Clot retraction.
Number: 150,000-400,000/mm3
Definition:
(Normal
Platelet platelet count
count <150.000/mm3
= 150.000–
400,000 /mm3).
Causes:
Divided into 2groups according to
Megakaryocyte in the bone marrow:
1- Decrease Megakaryocyte in B.M:
MostAplastic
Common anemia.
causes of thrombocytosis in Egypt?
Malignent diseases:
a- 1ry in bone marrow e.g leukemia.
-Splenectomy
b- 2 ries e.g neuroblastoma.
Megaloblastic Anemia
DIC
Repated Transfusion of stored Bl.
Definition:
Anti-platelet Ab is IgG
Immune disorder due to destruction of the
prepheral platelets by antibodies. Usually it
-Specie specific
follows viral upper respiratory
-Can cross tract thrombocytopenia
placenta causing infection by 2
-Active against homologous and
weeks. autologus platelets
Usually it is acute & self limiting but if it persist
>6 months it chronic.
Incidence:
The most common cause of thrombocytopenia
Female= male
Common from 2 – 8 years
Char
a c te
rs of
ITP
Platelet
dysfunction
Congenital
Petechiae
Purpura
Ecchymosis
Neonates Acquired
Low platelet count Infants
(Thrombocytopenia) Children
circulation.
Treatment
Conservation treatment: Because the disease is
self-limiting, the patient is put under observation
for fear of intracranial hemorrhage.
Corticosteroid:
Prednisone→ 60mg/m2/day, oral, for 2-3weeks & then
stop bleeding .
release Ristocetin
reactions
Platelet Bleeding
time
count
Acquired vascular condition
Henoch-Schollein purpura
Normal Purpura factitia
Clinical approach to nonthrombocytopenic Purpura
based on bleeding time
Clot formation results from interaction
between 13 coagulation factors
Factor I Fibrinogen
Factor II Prothrombin
The Coagulation factors are
Factor III Thromboplastin
.
Factor IV Calcium
Factor V Labile factor((Proaccelerin
Plasma thromboplastin
Factor XI
antecedent factor
It occurs through
3 phases
Phase I
1. Intrinsic pathway
2. Extrinsic pathway
w a y
a t h
i c p
r i n s
I n t
--Resposible for formation of plasma
thromboplastin.
X Xa Platelet
aggregation
XIII
V, Va, Ca++ , PF3
Prothrombin Thrombin
Fibrin Fibrin
Fibrinogen monomers polymers
IXa IX Platelet
plug
V, Va, Ca++ , PF3 Loose
X Xa Platelet
aggregation
XIII
VIII, VIIIa, Ca++ , PF3
Prothrombin Thrombin
Fibrin Fibrin
Fibrinogen monomers polymers
It i s s p e c i f i c
p ro t h r o m b i n
test for
time
Phase III
It is specific
t f o r t hr o m b in
tes
:(Time (TT
Normally
c o n d s 1 5 - 2 0
se
Hemophilias
A, B and C
Hemophilia A
So,
-- it affects males only
--females are carriers
Degrees:
Severe -------- Factor VIII < 1%
Moderate ------- Factor VIII 1-5%
Mild -------- Factor VIII 5-25%
Clinical Picture
1. Bleeding from:
Umbilical stump
CNS hemorrhage in neonates.
limping
limitation of movement due to ankylosis
General
Replacement
measures Hemoarthrosis
therapy
Treatment
1. General measures:
--- Avoid
---Trauma
---I.M injections
---Surgical procedures.
--- Local measures
---Pressure bandage
---Cold compresses
---Application of thrombin
preparations on small wounds
Treatment
2. Hemoarthrosis:
2. Cryoprecipitate.
Each unit contain 80-100 IU of factor VIII.
o a r t hr os i s is
He m
le s s c o m m o n
e
Than in A typ
Hemophilia C
X- linked recessive
(inherited like hemophilia A)
Characterized by: