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1. Bleeding Disorders Caused
By Vascular Abnormalities
Dr Radfan Saleh

2. Abnormal bleeding may result from :
1. Vascular disorders
2. Thrombocytopenia
3. Defective platelet function
4. Defective coagulation
vascular & platelet disorders tend to be associated
with bleeding from mucous membranes & into
the skin, whereas
in coagulation disorders the bleeding is often into
joints or soft tissue

3. Vascular bleeding disorders:
 Are heterogeneous group of conditions
characterized by easy bruising & spontaneous
bleeding from the small vessels.
 The underlying abnormality is either in the :
 Vessels themselves
 Perivascular connective tissues

4. Vascular bleeding disorders:
 Most cases of bleeding caused by vascular defects
alone are not severe.
 Frequently the bleeding is mainly in the skin 
purpura , petechiae & ecchymoses …
 While in some other cases, there is more bleeding
from mucous membranes.
 In vascular bleeding disorders mainly all the
standard screening tests are normal including
platelet count, coagulation factors & bleeding time.

5. Vascular defects may be :
 Inherited
 Acquired ( more common )

6. Inherited vascular disorders :
 Hereditary Hemorrhagic Telangiectasia
 Ehlers Danlos syndrome

7. Hereditary Hemorrhagic Telangiectasia
 Uncommon disorder
 Autosomal dominant trait
 Pathogenesis :
 Presence of Telangiactasis (dilated, superficial blood
vessels that create small, focal red lesions) in the skin,
mucous membrane & internal organs.
 Telangiactasia maybe with pinpoint, nodular or
spiderlike appearance, fragile & tend to rupture.
 The lesions blanch when pressure is applied.
 The disorder usually becomes manifest by puberty &
progress throughout life

8. Clinical findings :
 Recurrent & severe Epistaxis (universal finding)
 Pulmonary arteriovenous malformations
 Recurrent oral cavity, gastrointestinal & urogenital
tract hemorrhage
 Iron deficiency anemia due to blood loss.

9. Hemorrhagic Telangiectasia

10. Diagnosis :
 The classic diagnostic triad :
 Family history
 Presence of telangiactasia .
 Bleeding from telangiactatic lesions .
 Coagulation factors, platelet count & Bleeding time
are normal .
 Tourniquet test may demonstrate increased capillary
fragility .

11. Ehlers Danlos Syndrome
 Rare connective tissue disorder
 with abnormal collagen formation .
 Autosomal dominant trait .
 This syndrome characterized by :
 Extreme vascular fragility
 Skin hyper elasticity
 Hyper extensible joints

12. Ehlers Danlos Syndrome

14. Clinical findings :
 Bleeding may clinically manifest as a result of
increased fragility of subcutaneous tissues
 Petechiae & purpura
 Easy bruising
 Gastrointestinal & gingival bleeding

15. Laboratory findings :
 Abnormal platelet adhesion & aggregation
 Prolonged bleeding time

16. Good luck
Dr.Radfan
/10/2020