VASCULAR PURPURAS
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Hemostasis
• Haemostasis refers to spontaneous arrest
of bleeding caused by injury of small
blood vessels.
• Small vessels are continuously exposed
to trauma during normal activities can not
bleed due to effects of haemostasis.
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Hemostasis - Functions
• Maintains blood in a fluid, clot free
state in normal vessel.
• Induce
rapid
and
localized
haemostatic plug at the site of injury.
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Hemostasis - Mechanism
Haemostasis depends on three components
• Vascular wall
• Platelets
• Coagulation cascade
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Hemorrhage and its Forms
• Hemorrhage refers to extravasation of blood
due to rupture of blood vessels. Haemorrhage
may be external or may be enclosed within a
tissue.
• Hematoma
means
accumulation
of
haemorrhagic blood in a tissue.
• Petechae is minute 1 to 2 mm haemorrhage
into skin, mucous membrane or serous surface.
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Hemorrhage and its Forms
• Purpura is haemorrhage larger than 3 mm into
skin and mucous membrane.
• Echymosis is subcutaneous haematoma larger
than 2 cm.
• Large accumulation of blood in body cavities
like pleural cavity, peritoneal cavity,
pericardial cavity, synovial cavity are called
–
–
–
–
haemothorax,
haemoperitoneum,
haemopericardium
haemarthrosis.
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Clinical Significance of
Hemorrhage
• Clinical effects of haemorrhage depend on
– volume and rate of blood loss - rapid removal of up to
20% of blood volume or slow losses of even larger
amount may have little impact in healthy adult.
Greater losses may result in haemorrhagic shock.
– site of haemorrhage is also important. Bleeding
that would be insignificant in subcutaneous tissue
may cause death if located in the brain because the
skull is unyielding and bleeding can result
increased intracranial pressure and herniated.
– chronic or recurrent external blood loss may lead
to iron deficiency anaemia.
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Hemorrhagic Disorders
Hemorrhagic disorders are a group of disorders
of many different causes characterized by an
abnormal tendency of bleeding due to failure
of hemostasis.
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Clinical Character of
Hemorrhagic Disorders
• Spontaneous bleeding in the skin, mucous
membrane or internal tissue.
• Extensive or prolonged bleeding following
trauma.
• Bleeding from more than one site.
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Causes of Hemorrhagic
Disorders
•
•
•
•
Due to vascular defects
Due to platelet defects
Due to coagulation defects.
Combination of all
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Bleeding due to vascular defects
Common characters:
• More common but less severe
• Usually in the form of petechae or purpura
• Increased bleeding time and frequently
positive tourniquet test.
• Normal platelet count or function.
• Normal CT, APTT, PT and TT
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Vascular Disorders Classification
Acquired:
• Simple easy bruising (devil’s
pinches)
• Senile purpura
• The symptomatic vascular
purpura (non-thromcytopenic
purpura)
– Infection
– Drugs
– Uraemia
– Cushing’s syndrome and
adrenocorticosteroid
administration
– Scurvy
– Dysproteinaemia
– Henoch-Schoenlein purpura
• Miscellaneous
– Orthostatic purpura
– Mechanical purpura
– Fat embolism
– Auto-erythrocyte sensitization
– Systemic disorders – collagen
diseases, polyarteritis nodusa,
amyloidosis, allergy
Congenital:
• Hereditary haemorrhagic
telangiectasis (Osler-Renduweber disease)
• Ehlars-danlos disease
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Henoch-Schönlein Purpura
(HSP)
HSP - Background
 HSP is a small-vessel vasculitis characterized by
purpura, arthritis, abdominal pain, and hematuria.
 It is also known as anaphylactoid pupura.
 It is the most common cause
thrombocytopenic purpura in children
of
non-
HSP - Epidemiology
• The etiology of HSP is unknown, but it typically
follows as upper respiratory tract infection.
• It used to account for about 1% of the admissions to
hospital but changes in the medical practice have
reduced the frequency to about 0.6%.
• It is more frequent in children more than adult, and
mostly occurs between the ages of 2-8 years.
HSP - Epidemiology
• Males are affected twice as frequently as
females.
• And the overall incidence is estimated to be
9/100000 population
HSP - Etiology
 The etiology of HSP is unknown.
 About 50% of patients have a preceding upper respiratory
illness (URI).
 Multiple infectious agents as well as drugs, foods, and
insect bites may trigger HSP.
HSP - Pathophysiology
 This illness is considered by histopathology to be
an IgA mediated vasculitis of small vessels.
 And immunoflorescent techniques may show
deposition of IgA and C3 in the small vessels of
the skin and the renal glomeruli
HSP - Pathophysiology
 HSP and IgA nephropathy are related disorders.
 Both illnesses have elevated serum IgA levels and identical
findings on renal biopsy.
 however, IgA nephropathy almost exclusively involves
young adults and predominantly affects the kidneys only.
 HSP affects mostly children and involves the skin and
connective tissues, gastrointestinal tract, joints, and
scrotum as well as the kidneys.
HSP - Mortality/Morbidity
 In general, HSP is a benign self-limited disorder.
 Fewer than 5% of cases cause chronic symptoms.
 Fewer than 1% of cases progress to end-stage renal failure.
 Sex: Male-to-female ratio is about 2:1.
 Age: Seventy-five percent of patients affected are aged 2-8 years.
HSP - History
• The onset of the disease maybe acute with the
appearance
of
several
manifestations
simultaneously, or insidious with sequential
occurrence of symptoms over period of weeks
to months.
• Low grade fever and fatigue occur in more
than half of affected children
HSP – Cinical Manifestations
 Rash (95-100%), especially involving the legs, may
not be present on initial presentation
 Beginning as pinkish maculopapules that initially
blanches on pressure.
 Then progress petechiae or purpura which are
characterized clinically as palpable pupura that evolve
from red to purple rusty brown before they eventually
fade.
HSP – Cinical Manifestations
 The lesions tend to occur in crops lasting from 310 days and may appear at intervals that vary from
a few days to as long as 3-4 months.
 In fewer than 10% of children, recurrence of the
rash may not end until as late as a year and rarely
several years after the initial episode
Purpura
Purpura
HSP – Cinical Manifestations
 Subcutaneous edema (20-50%)
 Damage to cutanuous vessels also may result in local
angiodema, which may preceed the palpable purpura.
 Edema occurs primarily in dependent areas, as below
the waist, over the buttocks or on the back and
posterior scalp of infants, or in areas of greater tissue
destisibility such as eyelids, lips, scrotum, or dorsum
of the hands or feet.
HSP – Cinical Manifestations
 Joint pain (60-80%), especially involving knees and
ankles
 Arithritis is usually localized to the knees and ankles and
appears to be concomitant with edema.
 The effusion are serous, not hemorrhagic, in nature and
resolve after few days without residual deformity or
articular damage.
 They may recur during a subsequent reactive phase of the
disease
HSP – Cinical Manifestations
 Abdominal pain and vomiting (85%)
 Edema and damage to the vasculature of the GIT may also
lead to intermittent abdominal pain that is often colicky in
nature.
 More than half of patients have occult positive stools,
diarrhea or hematemesis.
 The recognition of peritoneal exudates, enlarged mesenteric
lymph nodes, segmental edema, and hemorrhage into the
bowel may prevent unnecessary laporotomy for acute
abdomen.
 Intussusception may occur, which is suggested by an empty
right lower abdominal quadrant on physical examination or
by cuurant jelly stools, which may be followed by complete
obstruction or infarction with bowel perforation.
HSP – Cinical Manifestations
• Several other organs may be involved during the acute phase of
the disease.
• Renal involvement occur in 20-50% of patients.
• Hepatosplenomegaly and lymphadenopathy may also occur.
• A rare but potentially serious outcome of the CNS involvement
is the development of seizures, paresis, or coma.
• Other rare complications include cardiac and eye involvement,
mononeuropathy, pancreatitis and pulmonary or intramuscular
hemorrhage
HSP – Physical
 Palpable purpura, particularly on the buttocks and legs
Edema of the hands, feet, scalp, and ears
 Arthritis, most commonly involving the knees and ankles
 Abdominal tenderness
 Gastrointestinal bleeding
 Acute scrotal edema that may mimic testicular torsion
HSP - Lab Studies
 Platelet count and coagulation studies:
 Platelet count is usually in the reference range but may be
elevated; the platelet count should not be low in HSP.
 A normal platelet count rules out idiopathic thrombocytopenic
purpura (ITP).
 A normal platelet count and normal coagulation studies (ie, PT,
aPTT, fibrin split products) rule out thrombotic
thrombocytopenic purpura (TTP).
 Lipase: A normal lipase makes acute pancreatitis very
unlikely.
HSP - Lab Studies
 CBC and differential: WBC count may be in the reference
range or elevated. Eosinophilia is sometimes present.
 Erythrocyte sedimentation rate: Sedimentation rate may
be in the reference range or elevated.
 BUN and creatinine levels: These may be elevated from
renal involvement of HSP or from dehydration.
 Urinalysis: Hematuria and/or proteinuria are present
in 10-20% of patients.
• Definitive diagnosis of vasculitis, confirmed by biopsy of
an involved cutanuous site, shows leuckocytoclastic
angiits.
• Renal biopsy may show IgA mesengial deposition and
occasionally IgM,C3, and fibrin.
HSP - Medications
• Nonsteroidal anti-inflammatory drugs (NSAIDs) may
help joint pain and do not worsen the purpura.
However, NSAIDs should be used cautiously in
patients with renal insufficiency.
• Clinicians often use corticosteroids to treat abdominal
pain, subcutaneous edema, and nephritis, but no
prospective,
placebo-controlled
studies
have
demonstrated their effectiveness. Most patients
recover quickly without treatment.
HSP - Medications
• Treatment in patients with severe HSP nephritis
includes many different modalities, but again, no
prospective controlled studies prove the efficacy of
any of these treatments.
• Treatment regimens have included IV or oral steroids
with or without any of the following: azathioprine,
cyclophosphamide, cyclosporine, high-dose IV
immunoglobulin G (IVIg), danazol, or fish oil.