This document discusses bleeding disorders caused by vascular defects. It defines different types of hemorrhages including petechiae, purpura, and ecchymoses. It describes acquired vascular disorders such as senile purpura, scurvy, and vasculitis. Inherited disorders discussed are hereditary hemorrhagic telangiectasia and Ehlers-Danlos syndrome. The diagnostic approach for vascular purpuras is outlined.

1. By :
Mohamed Ahmed

2. Outline
 Introduction to bleeding disorders
 Bleeding due to vascular causes

3.  Bleeding disorders is a general term for a group of
conditions (acquired or inherited) in which there is an
abnormal tendency to bleeding /hemorrhage due to
failure of hemostasis.
 The bleeding may be heavy and prolonged which may
occur after an insignificant injury or spontaneously.
 The pattern of bleeding is relatively predictable
depending on the aetiology.
 Vascular and platelet disorders tend to be associated
with bleeding from mucous membranes and into the
skin.
 whereas in coagulation disorders the bleeding is often
into joints or soft tissue

6.  Petechiae : are small (1 to 2 mm in diameter),
red to purple hemorrhagic spots in the skin,
mucous membranes or serosal surfaces. They
are most commonly found with low platelet counts
(thrombocytopenia) or defective platelet function.
 Purpura: it means purple. They are slightly larger
(> 3 mm) than petechiae. The causes are
thrombocytopenia, increased vascular fragility
and vasculitis.

7.  Ecchymoses: They are larger (>1 to 2 cm) and
result from blood escaping through endothelium
into intact subcutaneous tissue.

10. DISORDERS OF HEMOSTASIS
 Normal hemostasis requires a well controlled
collaboration among the five components of
hemostasis namely:
vascular endothelium, platelets, coagulation
system, coagulation regulatory system and
fibrinolytic system.

12. Clinical features
• Spontaneous bleeding in the skin, mucous
membrane or internal tissue.
• Extensive or prolonged bleeding following
trauma.
• Bleeding from more than one site.

13. Causes of bleeding disorders
• 1.Due to vascular defects
• 2.Due to platelet defects
• 3.Due to coagulation defects

14. ‰
‰
LABORATORY EVALUATION
 platelet count
 peripheral smear examination, bleeding time, and
PFA (Platelet function analyser)-100Test
 prothrombin time and
 activated partial thromboplastin time
 Bleeding Time (BT)
 Platelet adhesion studies
 Platelet aggregation studies
 Platelet aggregation studies

15. Bleeding due to vascular
defects
 The blood vessel is one of the component that is
involved in hemostasis. Any structural damage to
either the endothelium or the supporting collagen
may result in bleeding.
They are relatively common disorders and
usually do not cause serious bleeding and
induce small hemorrhages (petechiae and
purpura) in the skin or mucous membranes. The
platelet count, bleeding time and results of the
coagulation tests (PT, PTT) are usually normal.
Hess test is usually positive.

16. CLASSIFICATION OF VASCULAR
BLEEDING DISORDERS:
• INHERITED
• ACQUIRED

17. Bleeding due to vascular
defects
 Acquired disorders
 1. Due to decreased amount of connective tissue
 Senile purpura
 Scurvy
 Cushing syndrome and steroid therapy
 2. Due to vasculitis
 Henoch-Schönlein purpura
 Infections
 Drug reactions
 3. Associated with plasma cell neoplasms
 Amyloidosis
 4. Miscellaneous
 Simple easy bruising

18. Contd..
 Inherited disorders
 Hereditary hemorrhagic telangiectasia
 Ehlers-Danlos syndrome
 Marfan syndrome

19. 1-Acquired disorders
 Senile Purpura: It is age-related atrophy of
supporting connective tissue of blood vessels.
 Scurvy: Lack of vitamin C (scurvy) is associated
with microvascular bleeding resulting from
impaired formation of collagens.
 Cushing syndrome and steroid therapy: In
Cushing syndrome, protein-wasting due to
excessive corticosteroid production causes loss
of perivascular supporting tissue.
 Simple easy bruising is a common benign
disorder which occurs in otherwise healthy
women, especially those of child‐bearing age.

20. Senile Purpura

21. Scurvy

23. HENOCH-SCHÖNLEIN
PURPURA
 This is an immune complex disease in which purpura
results from vasculitis (due to deposition of IgA-
containing complexes).
 Predominant occurrence in children (2–8 years)
 Rapid onset
 •Skin: Palpable purpuric spots over extensor surfaces
of extremities, associated with urticaria; sometimes
haemorrhagic bullous and necrotic lesions occur.
 Abdominal pain
 Arthralgia
 Kidneys: Microscopic haematuria, proteinuria, acute
glomerulonephritis, or nephrotic syndrome.

26.  The disease is self-limited but recurrent episodes
are common. Chronic glomerulonephritis, and
CNS bleeding are rare complications.
 Diagnosis is based on typical clinical features and
exclusion of haematological causes of purpura.
 Glucocorticoids are sometimes administered for
symptomatic relief.

27. Infections
 Meningococcemia, septicemia, infective
endocarditis and several of the rickettsioses.
 Mechanisms include invasion of vessel, septic
emboli, immune complex vasculitis or result of
disseminated intravascular coagulation (DIC).
 Amyloidosis

28. Petechiae in Vasculitis
(Rocky Mountain Spotted Fever)

29. 2- Inherited Disorders
 Hereditary Hemorrhagic Telangiectasia
(Weber-Osler-Rendu syndrome)
• It is an autosomal dominant disorder of vascular
malformation characterized by dilated, tortuous,
thin walled blood vessels of the skin and mucous
membranes.
Clinical picture include epistaxis, bleeding from
tongue mouth and GIT.
Repeated bleeding may result in iron deficiency
anemia.

31.  Ehlers-Danlos Syndrome: It is a congenital
disorder of defective collagen synthesis
characterized by joint hyper mobility skin laxity
and easy bruising.
 The capillaries are poorly supported by
subendothelial collagen and result in bleeding
mainly in the form of ecchymoses.

33. Diagnosis of vascular purpuras
 Superficial, mild bleeding from skin and mucous
membranes
 Bleeding from skin more in dependent portions of
body
 Screening tests of haemostasis often normal
 Associated clinical features usually characteristic