Bleeding Disorders Dr. Farjah H.AlGahtani Assistant Professor of Internal Medicin,Consultant...
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Transcript of Bleeding Disorders Dr. Farjah H.AlGahtani Assistant Professor of Internal Medicin,Consultant...
Bleeding Disorders
Dr. Farjah H.AlGahtaniDr. Farjah H.AlGahtaniAssistant Professor of Internal Medicin,Consultant HematologyDirector of transfusin Medicin and Blood Bank Department,KSU
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
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
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.
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
Bleeding: Clinical Features
Local - Vs - General, spontaneous. .
Hematoma / Joint Bleeds- CoagSkin / Mucosal Bleeds – PLTwound / surgical bleeding – Immediate - PLT Delayed - Coagulation
PlateletCoagulation
Petechiae, Purpura Hematoma, Joint bl.
Vascular disorders:
Petechiae, purpura, ecchymosessenile purpuravitamin C deficiency (scurvy)Connective tissue disordersInfections – MeningococcusHenoch-Schonlein Purpura-Immu
Senile PurpuraSenile Purpura
Petechiae in Petechiae in VasculitisVasculitis
(Rocky Mountain Spotted Fever)(Rocky Mountain Spotted Fever)
Henoch-Schonlein purpuraImmune
disorderChildrenFollows
infectionPetechiae with
edema and itching.
Henoch-Schonlein purpura
20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.
Platelet Disorders - Features:
Mucocutaneous bleedingPetechiae, Purpura, Ecchymosis.
spontaneous bleeding after trauma
CNS bleeding (severe plt)Prolonged bleeding time (BT)
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)
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
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,
Platelet Aggregation CurvesPlatelet Aggregation Curves
Von-Willebrand Disease:Coagulation + PLT disorder:Congenital disorderDeficiency of vWF molecule Part of FVIII, Mediates platelet adhesionProlonged Bleeding timeLow Factor VIII & long aPTTMucocutaneous bleeding
Von-Willebrand Disease
vWF: F-VIII & PLT function.
Defective Platelet Adhesion
Skin Bleeding Prolonged Bleeding
time.Low Factor VIII levels.
Coagulation disorders: Deficiencies of Clotting factors
Onset - delayed after traumaDeep bleeding
Into joints - Hemarthroses Into deep tissues – Hematoma large skin bleed – Ecchymoses
Blood Coagulation & TestsBlood Coagulation & Tests
CT- Large hematoma of psoas muscleCT- Large hematoma of psoas muscle
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
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)
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
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
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)
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
Summary Hemostatic Disorders BT Plt PT PTT
Vascular Dis - - - -
PLT Disorder - - - -
Factor 8/9 *Congenital - - -
Vit K / Liver*Acquired - - -
Combined (DIC) -
Summary
Symptom Platelet Coagulation Petechiae Yes No
Sites Skin & Mucosa
Deep Tissue
Time Immediate Delayed
Ecchymoses/Hematomas
Yes Yes
CLL-Thrombocytopenia
Lymphoid cells
Smear cells
(Fragile)
Dengue Hemorrhagic fever
Platelet deficiency..
Thrombocytopenia-TTP
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.
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