A.
Anees
fathima
ACQUIRED BLEEDING DISORDERS
ETIOPATHOGENESIS
ACQUIRED BLEEDING DISORDERS INCLUDES :
Vitamin K deficiency
Liver disease
Disseminated intravascular coagulation ( DIC )
Immune thrombocytopenic purpura ( IPT )
Von Willebrand’s disease
Haemolytic - Uremic syndrome
VITAMIN K DEFICIENCY
• Vitamin K is a fat-soluble vitamin and requires bile for its
absorption.
• Vitamin K is required by the liver for the production of factors II,
VII, IX and X, protein C, and protein S.
Causes :
1. Inadequate stores: As in hemorrhagic disease of the
newborn (vitamin K levels are low and are due to lack of gut
bacteria and low concentrations of the vitamin in breast milk) and
severe malnutrition (especially when combined with antibiotic
treatment).
2. Defective absorption: Diseases that interfere with fat
absorption, eg., obstructive jaundice owing to the lack of
intraluminal bile salts, pancreatic disease or small bowel disease.
3. Oral anticoagulant drugs which are vitamin K
antagonists (warfarin therapy).
Deficiency manifests as bleeding/hemorrhagic state.
LIVER DISEASE
• Biliary obstruction results in
malabsorption of vitamin K and
therefore decreased synthesis of
factors II, VII, IX and X.
Also there are decreased in factor V
and fibrinogen.
• Dysfibrinogenemia.
• Thrombocytopenia.
• Functional abnormalities of platelets.
• Hypersplenism associated with portal
hypertension.
• DIC.
DISSEMINATED INTRAVASCULAR COAGULATION
▸ There is widespread deposition of
fibrin within blood vessels with
consumption of coagulation
factors and platelets occurs as a
consequence of many disorders
which release procoagulant
material into the circulation or
diffuse endothelial damage or
generalised platelet aggregation
DIC CAUSES :
PATHOGENESIS
• DIC may triggered by the entry of procoagulant material into circulation:
- Amniotic fluid embolism
- APML
- Premature separation of placenta
- Initiated by widespread endothelial damage and collagen exposure:
- Septicaemia
- Severe burns
• Widespread intravascular platelet aggregation
- Some bacteria, viruses and immune complexes may have direct effect
on platelets.
IMMUNE THROMBOCYTOPENIC PURPURA
• Immune (idiopathic) thrombocytopenic purpura
(ITP) is an autoimmune disorder characterised
by increased destruction of platelets by
autoantibodies directed against platelet
membrane GIb/Ill-a and GPIb/IX.
• ITP is an autoimmune disorder with formation of
anti platelet antibodies, directed against
membrane glycoproteins most often IIb/Ill-a or
Ib/IX of platelets.
• The anti-platelet antibodies can be
demonstrated in approximately 80% of patients
and are of the IgG type.
CAUSES:
Escherichia coli (E. coli) from contaminated food/drink
• Enterohemorrhagic E. coli (EHEC, serotype 0157:H7); may be
caused by other strains
• E. coli attaches to intestinal wall -› secretes Shiga-like toxin -›
absorbed by intestinal blood vessels -> attaches to immune
cells -› toxins from white blood cells (WBCs) bind to endothelial
cells of glomerular capillaries -› inhibition of protein synthesis
-› apoptosis -› many tiny blood clots form in kidneys
PATHOLOGY :
• Small blood clots in tiny blood vessels, mostly in kidneys -> RBCs
break down, kidney function decreases -> urea levels in blood
increase
• Triggered by bloody diarrhoea
‣ Diarrhoea-positive/D+ haemolytic uremic syndrome (HUS/typical HUS)
RISK FACTORS :
Children < five years old, people 75+ years old, genetic
predisposition to endothelial cell damage
Atypical haemolytic uremic syndrome
• D-hemolytic uremic syndrome
• No preceding diarrhoea
• Damage to endothelial cell lining of glomerular capillaries
from infections not related to diarrhoea, medication,
autoimmune causes
• Infants, children
• Streptococcus pneumoniae presents as
pneumonia/meningitis.
Von Willebrand’s
disease
Acquired causes of VWF deficiency.
• Hematologic disorders
• Lymphoproliferative disorders
• Myeloproliferative neoplasms including essential thrombocytosis
(ET), systemic lupus erythematosus (SLE) and other autoimmune disorders,
and cardiovascular disease
• Congenital heart disease
• Aortic stenosis
• Left ventricular assist device or extracorporeal membrane oxygenation
• Wilms tumor hypothyroidism
• Valproic acid and other medications