BY

MOHANNAD IBN HOMAID

Approach to bleeding Disorders

A few points

Content Structure

1st half 2nd half

Overview

Why is it important ? Why is it so confusing ?

Basic Science Clinical manifestations Laboratory tests

Basic Science Review

Blood is goldThe 2 arms of Heamostasis

Platelets Clotting Factors

Small Vessel Response To Injury

Normal Response

Always Goes Through the following: Vascular Phase Platelet Phase Coagulation Phase Fibronlytic Phase

Pointless ? Or useful ?

Vascular Phase

Not Very important for understandingVasoconstrictionTXA2 and Aspirin

Platelet Phase

Platelet Phase

Unfortunately Very important The following occurs:

Platelet Adhesion (vWF later) Platelet Release Reaction

ADP TXA2

Temporary Plug < < BLEEDING STOPS HERE Bleeding time

The Tile

Coagulation Phase

Coagulation Phase

VERY IMPORTANT and VERY CONFUSINGWhy is it Confusing ?

Not Tangible Coagulation Phase and 12 factors Cofactors Ca and PF3 Extrinsic vs intrinsic Vitamin K factors Anti-Thrombin 3 And last but not least….

THE ROMAN NUMBERS

Coagulation Cascade

What do you need to know ? Simple Steps : extrinsic vs intrinsic Content of both How to test them Where they are made ( liver ) Vitamin K AT-3

CONFUSING

THIS SLIDE HAS BEEN INTENTIONALLY LEFT BLANK

Extrinsic System: 7Intrinsic System: 12-11-9-8Final Common Pathway :10-5-2-1Vitamin K : 2-7-9-10AT-3 : 12 -11-10-9PTT vs PT

Coagulation Studies

Fibrinolytic Phase

Fibrinolytic Phase

Kinnnd of important but very easyTissue plasminogen ActivatorPlasminTest

Fibrin Degradation Products D-Dimer Assay

Back to the clinical world

Presentation of platelet Defects Blood leaks out of vessels Skin and mucosal surfaces Prolonged bleeding ( temporary plug plug )

Presentation Deep Tissue Bleeding Late Rebleeding ( permanent plug Defect )

Laboratory Test

Platlets Count Bleeding Time Aggregation Test

Clotting Factors PT and PTT Factor Assay

Fibrinolysis FDP D-Dimer

Platelet Disorders

Quantitative vs QualitativeThrombocytopeniaImmune Thrombocytopenic PrupuraBernard Soulier SyndromeGlanzmanns ThrombastheniaThrombotic thrombocytopenic Purpura

Thrombocytopenia

Pathology :Increase Destruction or decrease Productions > >

Clinical Features : depend on degreeLabs:Treatment:

ITP

Pathology : Auto antibodies Agains PlatletsClinical Features:Labs:Treatment:

Bernard Soulier Syndrome

Pathology :GP1B receptor Defiency Clinical Features:Labs:

Glanzmann Thromboasthenia

Pathology :GPIIb-IIIa DefiencyClinical Features:Labs:Treatment:

TTP

Pathology :UnkownClinical Features: Pentad : HUS + Fever

NeurologicalLabs:Treatment :Plasmapharesis

Diorders of Coagulations

HemophiliaVon Willebrand Disease

Hemophilia

Pathology :Factor 8 or 9Clinical Features:

Acute Hemoarthrosis Intracranial Bleeding Hematomas

Labs:Treatment:

Factor Replacement DDAVP

Von willebrand Disease

Function of vWF Made in platelets and endothelium Adhesion of platelets to exposed Collagen Protection of Circulating Factor 8

Pathology Deficiency of vWF Secondary decrease in Factor 8

Von Willebrand Disease

Pathology : MentionedClinical Features:Labs:Treatment:

1. DDAVP And factor concentrates

DIC

Pathology :Inappropriate Activation of platelets and clotting Factors due to : Sepsis ( 50%) Obstetric Complications Malignancy Trauma

Clinical Features:Labs:Treatment: ICU and supportive = Treatment

of underlying Cause

Questions