Bleeding Disorders

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Bleeding Disorders
Dr. Farjah H.AlGahtani Assistant Professor of
Internal Medicin,Consultant Hematology Director
of transfusin Medicin and Blood Bank
Department,KSU
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Hemostasis

• CBC-Plt
• BT,(CT)
• PT
• PTT

BV Injury
Damage/contact.
Neural
Contact
Coagulation Cascade
Platelet Aggregation
Blood Vessel Constriction
Primary hemostatic plug
Reduced Blood flow
Platelet Activation
Fibrin formation
Platelet study Antibody tests Factor Assay
Stable Hemostatic Plug
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HEMOSTASIS

• Primary Hemostasis
• Blood vessel contraction
• Platelet Plug Formation
• Secondary Hemostasis
• Activation of Clotting Cascade
• Deposition Stabilization of Fibrin
• Tertiary Hemostasis
• Dissolution of Fibrin Clot
• Dependent on Plasminogen Activation

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Classification

• Disorders of Blood vessels
• Scurvy, senile purpura, Henoch-Schonlein
  syndrome.
• Disorders of Platelets
• Thrombocytopenia ITP, TTP, HUS, DIC.
• Aspirin therapy, Thrombasthenia,
• Disorders of Coagulation
• Extrinsic, intrinsic, combined.
• Other disorders
• Post transfusion purpura, MPS, MDS.

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Tests of Hemostasis

• Screening tests
• Bleeding.T - 10m. Platelet BV function
• Prothrombin.T Extrinsic, aPTT Instrinsic
• Thrombin.T common path. (DIC)
• Specific tests
• Factor assays hemophilia.
• Tests of thrombosis TT, FDP, DDA,
• Platelet function studies
• Adhesion, Aggregation, Release tests.
• Bone Marrow study

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Bleeding Clinical Features

• Local - Vs - General, spontaneous . .
• Hematoma / Joint Bleeds- Coag
• Skin / Mucosal Bleeds PLT
• wound / surgical bleeding
• Immediate - PLT
• Delayed - Coagulation

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Platelet Coagulation
Petechiae, Purpura Hematoma, Joint bl.
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Vascular disorders

• Petechiae, purpura, ecchymoses
• senile purpura
• vitamin C deficiency (scurvy)
• Connective tissue disorders
• Infections Meningococcus
• Henoch-Schonlein Purpura-Immu

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Senile Purpura
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Petechiae in Vasculitis (Rocky Mountain Spotted
Fever)
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Henoch-Schonlein purpura

• Immune disorder
• Children
• Follows infection
• Petechiae with edema and itching.

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Henoch-Schonlein purpura
20y Male, fever, painful symmetric polyarthritis
for a day. During the next two days, edema and
palpable purpura developed.
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Platelet Disorders - Features

• Mucocutaneous bleeding
• Petechiae, Purpura, Ecchymosis.
• spontaneous bleeding after trauma
• CNS bleeding (severe ? plt)
• Prolonged bleeding time (BT)

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BLEEDING TIME vs. PLATELET COUNT
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Idiopathic Thrombocytopenic Purpura (ITP)

• Acute - children (post infection)
• Chronic - adults (? females, 20-40 yrs)
• autoimmune disorder
• antiplatelet antibodies (IgG)
• IgG coated platelets removed by spleen
• Usually ? megakaryocytes in BM

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Platelet dysfunction

• Inherited Disorders
• Bernard-Soulier disease
• large platelets, failure of adhesion
• Glanzmanns thrombasthenia
• normal size, failure of aggregation
• Acquired Disorders
• Drugs - Aspirin, Alcohol, Uremia,

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Platelet Aggregation Curves
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Von-Willebrand Disease

• Coagulation PLT disorder
• Congenital disorder
• Deficiency of vWF molecule
• Part of FVIII,
• Mediates platelet adhesion
• Prolonged Bleeding time
• Low Factor VIII long aPTT
• Mucocutaneous bleeding

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• Von-Willebrand Disease
• vWF F-VIII PLT function.
• Defective Platelet Adhesion
• Skin Bleeding
• Prolonged Bleeding time.
• Low Factor VIII levels.

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Coagulation disorders

• Deficiencies of Clotting factors
• Onset - delayed after trauma
• Deep bleeding
• Into joints - Hemarthroses
• Into deep tissues Hematoma
• large skin bleed Ecchymoses

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Blood Coagulation Tests
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CT- Large hematoma of psoas muscle
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Coagulation Disorders

• Laboratory findings
• Normal bleeding time Platelet count
• Prolonged prothrombin time (PT)
• deficiencies of II, V, VII, X
• Prolonged time (aPTT)
• all factors except VII, XIII
• Mixing studies - normal plasma corrects PT or
  aPTT

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Factor VIII Deficiency

• Classic hemophilia (hemophilia A)
• X-linked disorder (affects 1º males)
• Most common - severe bleeding
• Spontaneous hematomas lt 1, 5, 75
• Abnormal aPTT Intrinsic path.
• Diagnosis - factor VIII assay
• Treatment - factor VIII concentrate
• Cryoprecipitate (less desirable)

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Factor IX Deficiency

• Christmas disease (Hemophilia B)
• X-linked recessive disorder
• Indistinguishable from classic hemophilia (F
  VIII)
• Requires evaluation of factor VIII and IX
  activity levels to diagnose
• Treatment - factor IX concentrate
• Cryoprecipitate if factor IX unavailable

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Secondary Hemostatic Disorders

• Acquired coagulation disorder
• Vitamin K deficiency
• - neonates - decreased intestinal
• flora and dietary intake
• - oral anticoagulants (coumadin)
• - fat malabsorption syndromes
• Required for factors II, VII, IX, X
• Prolonged PT and aPTT

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Combined Primary and Secondary Hemostatic
Disorders (DIC)

• Disseminated Intravascular Coagulation
• Major pathologic processes -
• obstetric complications, neoplasms, infection
  (sepsis), major trauma
• Primary - platelet consumption
• (? bleeding time, ? platelets)
• Secondary - factor consumption
• (? PT, aPTT)

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Combined Primary and Secondary Hemostatic
Disorders

• Severe Liver Disease
• Primary - dysfunctional platelets and/or
  thrombocytopenia (? BT)
• Secondary - decrease in all coagulation factors
  except vWF (? PT, aPTT)
• Vitamin K will promote synthesis of factors II,
  VII, IX, X

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Summary Hemostatic Disorders

• BT Plt PT PTT
• Vascular Dis - ? - - -
• PLT Disorder - ? - ? - -
• Factor 8/9
• Congenital - - - ?
• Vit K / Liver
• Acquired - - ? -?
• Combined (DIC) ? ? -? ?

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Summary
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CLL-Thrombocytopenia
Lymphoid cells Smear cells (Fragile)
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Dengue Hemorrhagic fever
Platelet deficiency..
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Thrombocytopenia-TTP
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Summary

• Complex system to keep blood fluid
• To block leakage on injury.
• BV, PLT Coagulation
• Complex inhibitory mechanisms
• Complex thrombolysis mechanisms.
• Screening tests BT, CT (PT, aPTT)
• Special tests Factor assay, PLT function etc.

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Disorders of Hemostasis

• Vascular disorders
• Scurvy, easy bruising, Henoch-Schonlein purpura.
• Platelet disorders
• Quantitative - Thrombocytopenia
• Qualitative - Platelet function disorders
  Glanzmans
• Coagulation disorders
• Congenital - Haemophilia (A, B), Von-Willebrands
• Acquired - Vitamin-K deficiency, Liver disease
• Mixed/Consumption DIC

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