This lecture was conducted during the Nephrology Unit Grand Ground by a Sub-intern under Nephrology Division, Department of Medicine in King Saud University. Nephrology Division is NOT responsible for the content of the presentation for it is intended for learning and /or education purpose only.

BY MOHANNAD IBN HOMAID Approach to bleeding Disorders

A few points Content Structure  1 st half  2 nd half

Overview Why is it important ? Why is it so confusing ?  Basic Science  Clinical manifestations  Laboratory tests

Basic Science Review Blood is gold The 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 understanding Vasoconstriction TXA2 and Aspirin

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

VERY IMPORTANT and VERY CONFUSING Why 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: 7 Intrinsic System: Final Common Pathway : Vitamin K : AT-3 : PTT vs PT

Coagulation Studies

Fibrinolytic Phase

Kinnnd of important but very easy Tissue plasminogen Activator Plasmin Test  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 Qualitative Thrombocytopenia Immune Thrombocytopenic Prupura Bernard Soulier Syndrome Glanzmanns Thrombasthenia Thrombotic thrombocytopenic Purpura

Thrombocytopenia Pathology :Increase Destruction or decrease Productions > > Clinical Features : depend on degree Labs: Treatment:

ITP Pathology : Auto antibodies Agains Platlets Clinical Features: Labs: Treatment:

Bernard Soulier Syndrome Pathology :GP1B receptor Defiency Clinical Features: Labs:

Glanzmann Thromboasthenia Pathology :GPIIb-IIIa Defiency Clinical Features: Labs: Treatment:

TTP Pathology :Unkown Clinical Features: Pentad : HUS + Fever Neurological Labs: Treatment :Plasmapharesis

Diorders of Coagulations Hemophilia Von Willebrand Disease

Hemophilia Pathology :Factor 8 or 9 Clinical 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 : Mentioned Clinical 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