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Bleeding Disorders
Dr. Farjah H.AlGahtaniDr. Farjah H.AlGahtaniAssistant Professor of Internal Medicin,Consultant HematologyDirector of transfusin Medicin and Blood Bank Department,KSU
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Hemostasis
BV Injury
PlateletPlateletAggregation
PlateletActivation
Blood VesselBlood Vessel Constriction
CoagulationCoagulation Cascade
Stable Hemostatic Plug
Fibrin formation
Reduced
Blood flow
Damage/contact.
Primary hemostatic plug
Neural
•CBC-Plt•BT,(CT)•PT•PTT
Platelet studyAntibody testsFactor Assay
Contact
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HEMOSTASISPrimary 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- CoagSkin / Mucosal Bleeds – PLTwound / surgical bleeding – Immediate - PLT Delayed - Coagulation
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PlateletCoagulation
Petechiae, Purpura Hematoma, Joint bl.
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Vascular disorders:
Petechiae, purpura, ecchymosessenile purpuravitamin C deficiency (scurvy)Connective tissue disordersInfections – MeningococcusHenoch-Schonlein Purpura-Immu
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Senile PurpuraSenile Purpura
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Petechiae in Petechiae in VasculitisVasculitis
(Rocky Mountain Spotted Fever)(Rocky Mountain Spotted Fever)
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Henoch-Schonlein purpuraImmune
disorderChildrenFollows
infectionPetechiae 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 bleedingPetechiae, Purpura, Ecchymosis.
spontaneous bleeding after trauma
CNS bleeding (severe plt)Prolonged bleeding time (BT)
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BLEEDING TIME vs. PLATELET COUNT
0
50
100
150
200
250
300
350
400
3.5 4 4.5 5 5.5 7 9 12 15 25 30
Minutes
Pla
tele
t co
un
t (x
100
0)
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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 spleenUsually megakaryocytes in BM
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Platelet dysfunction:Inherited Disorders: Bernard-Soulier disease
large platelets, failure of adhesionGlanzmann’s thrombasthenia
normal size, failure of aggregation
Acquired Disorders:Drugs - Aspirin, Alcohol, Uremia,
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Platelet Aggregation CurvesPlatelet Aggregation Curves
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Von-Willebrand Disease:Coagulation + PLT disorder:Congenital disorderDeficiency of vWF molecule Part of FVIII, Mediates platelet adhesionProlonged Bleeding timeLow Factor VIII & long aPTTMucocutaneous 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 traumaDeep bleeding
Into joints - Hemarthroses Into deep tissues – Hematoma large skin bleed – Ecchymoses
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Blood Coagulation & TestsBlood Coagulation & Tests
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CT- Large hematoma of psoas muscleCT- Large hematoma of psoas muscle
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Coagulation DisordersLaboratory findings:Normal bleeding time & Platelet countProlonged 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 DeficiencyClassic hemophilia (hemophilia A)X-linked disorder (affects 1º males)Most common - severe bleedingSpontaneous hematomas < 1, 5, 75%Abnormal aPTT – Intrinsic path.Diagnosis - factor VIII assayTreatment - factor VIII concentrateCryoprecipitate (less desirable)
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Factor IX DeficiencyChristmas disease (Hemophilia B)X-linked recessive disorder Indistinguishable from classic
hemophilia (F VIII)Requires evaluation of factor VIII and IX
activity levels to diagnoseTreatment - factor IX concentrateCryoprecipitate 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
Symptom Platelet Coagulation Petechiae Yes No
Sites Skin & Mucosa
Deep Tissue
Time Immediate Delayed
Ecchymoses/Hematomas
Yes Yes
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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 fluidTo block leakage on injury.BV, PLT & CoagulationComplex inhibitory mechanismsComplex 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 –
GlanzmansCoagulation disorders
Congenital - Haemophilia (A, B), Von-Willebrands
Acquired - Vitamin-K deficiency, Liver diseaseMixed/Consumption: DIC
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