haemostasis Vascular bleeding disorders The underlying abnormalities are either in the vessels themselves perivascular connective tissue Each could be Inherited Acquired autosomal ID: 916324
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Slide1
Haematology
Slide2Haemostasis
Slide3The involvement of blood vessels, platelets and blood coagulation in haemostasis
Slide4Vascular bleeding disorders:
.The underlying
abnormalities are either in the vessels themselves
perivascular
connective tissue
.
Each could be
Inherited
Acquired
autosomal
dominant trait.
There are dilated microvascular swellings appear during childhood and become more numerous in adult life.
These
telangiectasia
develop in the skin, mucous membranes and internal organs
Inherited
vascular
disorders
Hereditary
haemorrhagic
telangiectasia
Slide7Slide8hereditary collagen abnormalities
with
purpura resulting from defective platelet aggregationhyperextensibility of joints and hyperelastic friable skin
Ehlers-
Danlos
syndrome
Slide9Simple
easy bruising
Purpura associated with infectionsSenile
purpra
Scurvy
The
Henoch-Schonlein
syndrome
Steroid
purpura
Acquired vascular defect
Slide10Slide11a
b
Slide12immune complex (typeIII
) hypersensitivity reaction usually found in children often following acute infection
The characteristic purpuric rash accompanied by localized edema & itching is often most prominent on the buttocks &extensor surfaces of lower legs &
elbows
Painful joint swelling,
haematuria
& abdominal pain may also occur.
Henoch
–
Sconlein
syndrome
Slide13Thrombocytopenia
Slide14Slide15Slide163-Abnormal distribution of platelets
*
splenomegaly4-Dilutional loss*Massive transfusion of stored blood
Slide17Slide18Hereditary disorders
Thrombasthenia
(Glanzmann's disease)
Bernard-
Soulier
syndrome
Storage pool diseases
Acquired disorders
Antiplatelet
drugs
Hyperglobulinaemia
Myeloproliferative
and
myelodysplastic
Disorders
Uraemia
Heparin,
dextrans
, alcohol and
radiographic contrast
Slide19Slide20Slide211.Clinical assessment.
2.Labroratory assessment.
Assessment of Coagulation
Slide22Bleeding time:
Platelet counts:
Prothrombin time (PT):Partial thromboplastin time (PTT):
Thrombin time (TT):
Laboratory assessment of Coagulation:
Slide23Slide24A 5 yr old boy presents with a haemarthrosis of the right knee. Coagulation studies show normal PT ,normal bleeding
time,normal
TT, prolonged APTT .Explain the coagulatin defect?
Case
Slide25Slide26Slide27The defect is in the intrinsic pathway .
Haemophillia
A or
Haemophillia
B
Factor assay.
Slide28Haematoma
Slide29DIC
Slide301 .The platelet count is low.2. Fibrinogen concentration low.
3 .The thrombin time is prolonged.
4 .High levels of fibrin degradation products such as D -dimers are found in serum and urine.5. The PT and APTT are prolonged . 6. bleeding time is prolonged.
Lab. Findings:
Slide31THANK
YOU