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Bleeding disorders

The document provides a comprehensive overview of bleeding disorders, including classifications such as vascular causes, platelet-related causes, coagulation disorders, and fibrinolytic system defects. It outlines the clinical approach to diagnosing these disorders, including when to work-up, the nature of bleeding episodes, and necessary laboratory tests. Additionally, it details the interpretation of lab results to identify specific deficiencies or disorders.

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jhajharia.niharika
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9 views

Bleeding disorders

The document provides a comprehensive overview of bleeding disorders, including classifications such as vascular causes, platelet-related causes, coagulation disorders, and fibrinolytic system defects. It outlines the clinical approach to diagnosing these disorders, including when to work-up, the nature of bleeding episodes, and necessary laboratory tests. Additionally, it details the interpretation of lab results to identify specific deficiencies or disorders.

Uploaded by

jhajharia.niharika
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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Bleeding Disorders

Low platelets?
Coagulopathy?
Platelet dysfunction!!?!! Overview
•Clinical approach
i ng So m
b
d
lee ? know any cause •Baseline lab-work
t a rder of! W s
Is i o hich I
dis is it? one •Special tests

h i l d! •Flow sheet for approach


i ngc
l e ed
B
Primary hemostasis Secondary hemostasis Regulation

Local vasoconstriction Coagulation system


Endothelial collagen exposure -Activation of extrinsic pathway
Interaction: collagen, vWF, -Activation of intrinsic pathway
platelet -> platelet plug Anticoagulant proteins

Unstable clot Stable clot


CLASSIFICATION OF
HEMORRHAGIC DISORDERS

• Vascular causes
• Platelet related causes
• Coagulation disorder
• Fibrinolytic disorders
CLASSIFICATION OF HEMORRHAGIC DISORDERS

(1.) Vascular causes


Hereditary
• Hereditary hemorrhagic telengiectasia (osler-Rendu-Weber disease)
• Hereditary capillary fragility
• Ehler’s Danlos syndrome
• Marfan syndrome

Acquired
• Infections – Typhoid fever, SABE, meningococcal septicemia, purpura
fulminans, infectious mononucleosis.
• Drugs – Penicillins, sulfonamides, hydantoin, aspirin, barbiturates,
insulin, isoniazid, chlorpromazine, streptomycin.
• Uremia
• Scurvy
• Henoch Schonlein purpura
CLASSIFICATION OF HEMORRHAGIC DISORDERS

(2.) PLATELET RELATED


1. QUANTITATIVE DEFECT (Thrombocytopenia)

• Decreased Production : marrow invasion, cytotoxic drugs,


aplasia, nutritional (Folate, B12 deficiency), TAR syndrome,
Fanconi’s aplastic anemia. Bernard soulier syndrome (BSS),
wiskott Aldrich syndrome.
• Sequestration : hypersplenism, hemangioma.
• Increased platelet destruction : ITP, SLE, drug induced.
• Increased consumption : DIC, HUS, TTP, .
CLASSIFICATION OF HEMORRHAGIC DISORDERS

(2.) PLATELET RELATED

2. QUALITATIVE DEFECTS

• Adhesion – von willebrand’s disease, giant platelet


syndrome (BSS), uremia.
• Aggregation – Glanzman’s thrombosthenia, drug induced
(aspirin), afibrinogenemia, dysproteinemias.
• Disorders of granule release – storage pool disease, drug –
aspirin & other NSAIDs, chediak higashi syndrome.
CLASSIFICATION OF HEMORRHAGIC DISORDERS

(3.) COAGULATION DISORDERS

Hereditary
(i) hemophilia A,B,C,
(ii) factor I, II, V, VII, X, XIII deficiency.

Acquired
(i) Vitamin K deficiency
(ii) Liver disease
(iii) Anticoagulant therapy
(iv) DIC
(v) Inhibitors
CLASSIFICATION OF HEMORRHAGIC DISORDERS

(4.) FIBRINOLYTIC SYSTEM DEFECT

• Primary pathological fibrinolysis

• Disseminated intravascular coagulation


APPROACH TO A CASE OF BLEEDING DISORDER

When to Work-Up

• When bleeding episode- spontaneous or after


injury/surgery is prolonged and disproportionate to the
situation.
• Family history of bleeding episodes.
• Before surgery and invasive procedures.
• Systemic diseases known to be associated with bleeding
disorder e.g. liver disorder, DIC, sepsis, etc.
APPROACH TO A CASE OF BLEEDING DISORDER

In a bleeding episode decide:

• Is bleeding significant ? Yes


• Whether it is due to local cause or generalised
haemostatic defect.
• Is it acquired or hereditary
• What is nature of bleeding episode ?
- vascular/platelet/coagulation defect/mixed
APPROACH TO A CASE OF BLEEDING DISORDER

Answers to these questions require

• A detailed history including past, family and drug


history
• Detailed physical examination
• Screening tests
• Specific tests as needed
APPROACH TO A CASE OF BLEEDING DISORDER

Q 1. Is bleeding due to :

• Local cause or
• Generalized disorder of haemostasis
APPROACH TO A CASE OF BLEEDING DISORDER

Local cause :
- more common
- bleeding generally happens from single site with
recurrence often from same site
e.g. Epistaxis (dryness, AR, adenoids, nose picking)
?? Requiring ER visit / both nostrils / other
associated bleeds / past history or family
history of bleeding
APPROACH TO A CASE OF BLEEDING DISORDER

Bleeding in generalized disorder :

• spontaneous and recurrent


• disproportionate to extent of injury
• bleeding may be seen in skin in form of
petechiae, purpura
• deep tissue bleeding
APPROACH TO A CASE OF BLEEDING DISORDER

Q 2. Is bleeding due to

• Congenital disorder
• Acquired disorder
APPROACH TO A CASE OF BLEEDING DISORDER

Congenital
Early presentation

• Circumcision bleeding, umbilical stump bleeding,


cephalhaematomas/subgaleal haemorrhages
without difficult labour, bleeding during eruption and
fall of teeth
• ?? Milder forms may present later
APPROACH TO A CASE OF BLEEDING DISORDER

Congenital
Heritable :
• X linked : haemophilia (30% new mutations), Wiscott
Aldrich synd.
• Autosomal dominant : Von Willebrand disease
dysfibrinogenemia, hereditary haemorrhagic
telangiectasia
• Autosomal recessive : Deficiency of factors 2,5,7,10,
11,12,13,prekallikrein,HMWK
APPROACH TO A CASE OF BLEEDING DISORDER

Acquired
• Usually present later in life
• Negative family history
• There may be underlying systemic disorders
like liver diseases, infections etc.
APPROACH TO A CASE OF BLEEDING DISORDER

Q 3. What is nature of bleeding episode :

• Vascular / Platelet
• Coagulation disorder
• Mixed
APPROACH TO A CASE OF BLEEDING DISORDER
APPROACH TO A CASE OF BLEEDING DISORDER

Laboratory tests
To assess:

• Integrity of vascular and platelet phase of


haemostasis
• Coagulation system
• Fibrinolytic pathway
CLOTTING CASCADE
APPROACH TO A CASE OF BLEEDING DISORDER

Baseline investigations
Platelet

• Complete blood count


• Peripheral smear (platelet no & size)
• Bleeding time ( in qualitative as well as quantitative
platelet defects)
• PFA
Coagulation

• Prothrombin time (I, II, V, VII, X)


• Activated partial thromboplastin time
(I, II, V, VIII, IX, X, XI, XII, pre-kallikrien, HMWK)
• Thrombin time (I)
APPROACH TO A CASE OF BLEEDING DISORDER

Special confirmatory tests

For platelet disorders

• Platelet function tests


Needed when APC is normal but bleeding time is
abnormal
APPROACH TO A CASE OF BLEEDING DISORDER

For coagulation disorders

• Whole blood clotting test (snake envenomation)


• Mixing study
• Factor assays
• Russel viper time (lupus anticoagulant)
• Reptilase Time (heparin contamination)
• Clot solubility in 5M Urea (factor XIII deficiency)
APPROACH TO A CASE OF BLEEDING DISORDER

Tests for fibrinolysis

• Euglobulin clot lysis time (ECLT)


• Fibrin degradation products
• D- dimer
• Fibrinogen
• Fibrinopeptide – A
• Plasmin, plasminogen
Interpretation of lab tests
APTT – Prolonged
PT – N
APC - N

Bleeding No Bleeding
• Von willebrand disease (by • Factor 12 deficiency
binding to factor 8 prevents its
degradation by protein C) • Deficiency of
• Deficiency of factor 8, 9 prekallikrien & HMWK
and 11 (intrinsic pathway) • Lupus anticoagulant
PT – Prolonged
APTT – N
APC - N

• Early vitamin K deficiency


• Early liver disease
• Factor 7 deficiency (Extrinsic pathway)
PT – Prolonged
APTT – Prolonged
TT - N
APC - N

• Vitamin K deficiency (needed to make factors 2,7,9 & 10)


• Liver disease (needed to make majority of clotting factors)
• Oral anticoagulant therapy (vitamin k antagonism)
• Deficiency of factors 10, 5 and 2 (common pathway)
PT – Prolonged
APTT – Prolonged
TT – Prolonged
APC - N

• Liver disease
• Heparin therapy (activates anti thrombin)
• Deficiency of factor 1 (fibrinogen)
PT – Prolonged
APTT – Prolonged
TT - Prolonged
APC - Decreased

• DIC
• Advanced liver disease ( thrombopoietin)
Bleeding and
PT – N
APTT – N
APC - N
Bleeding time – N Bleeding time

• Factor 13 deficiency ( 5 M urea • Von willebrand disease


clot solubility test ) (binds platelets to vessel wall )
• Excessive fibrinolysis • Other platelet function
(euglobulin clot lysis test )
disorders
• Hereditary hemorrhagic
telangiectasia
• Scurvy
• vasculitis

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